10⼀Heme-Onc/TOX I Flashcards

(383 cards)

1
Q

60

⬜ are 4 major triggers of vasooclusive crisis in Sickle Cell Disease patients

What is the treatment for vasooclusive crisis? (5)
_________________

Which med is used for long term management?

A

109DICK (Dehydration / Infection / Cold temp / [Kant breathe (hypoxia)])
_________________

acute tx = Rehydration / [abx and PAIN CONTROL (NSAID>opioids)] / heat / oxygen
_________________

chronic tx = Hydroxyurea

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2
Q

Which drugs cause Agranulocytosis? (6)

A

Gangs Can Certainly Crush Myeloblast & Promyelocytes

Ganciclovir

Clozapine

Carbamazepine

Colchicine

Methimazole(also Teratogenic–>Cutis Aplasia)

PTU

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3
Q

Victims of [smoke inhalation injury] should empircally be treated for Carbon Monoxide and what other chemical toxicity?

________________

What are the treatments for these toxicities? (4)

A

CYanide toxicity (➜ SEVERE lactic acidosis)

________________

“CYaMonoxide toxicity Needs Overt Smoke Help”

empiric tx for CYanide and (Carbon)Monoxide toxicity

  1. [Nitrites (induces methemoglobinemia)]
  2. Oxygen 100% (CO tx)
  3. [Sodium thiosulfate]
  4. HydroxoCobalamin (binds Cyanide ➜ excretable Cyanocobalamin)

{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}

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4
Q

Pulse Ox measures ⬜ which = ⬜

What does [PulseOx] read during methemoglobinemia?

________________

why?

A

**POSOSOP**
<sub>**P**ulse **O**x =</sub> **S**<sub>p</sub>**O**<sub>2 = </sub> [**S**aturation of **O**<sub>2</sub> on **P**ulsatile Peripheral arterial <sub>RBC</sub>]

* * *

**{low SpO2 < 85%}** = low **[**PulseOx<sup> </sup>S<sub>p</sub>O<sub>2_</sub>**RBC O2 Saturation**]<sub> </sub>\< 85%**}**

\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

⚠️*NOTE*: Even though [PaO<sub>2</sub> *oxygen partial pressure*] may read "normal", methemoglobin has lower affinity to O2 than Hgb = Mgb low binding to O2 ➜ **low [RBC O2 saturation]** = **{low POSOROS** = **[**PulseOx<sup> </sup>S<sub>p</sub>*O<sub>2_</sub>**Saturation*]<sub> </sub>\< 85%**}**

\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

*Dark chocolate colored blood*

## Footnote

[🔎SpO2 = Saturation of O2 on **P**ulsatile-Peripheral arterial RBC (via PulseOx)]
[ 🔎SaO2 = Saturation of O2 on **a**pulsatile (Non-pulsatile) arterial RBC(via ABG) <sub>= more accurate</sub>]

🫁<sub>**PaO2<sub>*ABG*</sub>** specifically refers to pressure solely exerted by dissolved oxygen in the arterial blood. It represents amount of oxygen actually dissolved in the plasma of arterial blood, rather than the amount that is bound to hemoglobin (which is reflected in measures like **SaO2<sub>*ABG*</sub>** or **SpO2<sub>*PulseOx*</sub>**).</sub>

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5
Q

Dark chocolate colored blood = ⬜

________________

MOA for tx? (3)

A

Methemoglobinemia

________________

1st: [NADPH gives electron to [METHYLENE BLUE] ]
2nd: this converts [METHYLENE BLUE] –> [LeukoMethylene blue]
3rd: [LM reduces Methomoglobin –> back to Hgb]

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6
Q

s/s of [hypOcalcemia < 0.76 iCal] (7)

A

TQT + BOD + Chvostek
_________________
low calcium tx = IV CaGLUCONATE or CaChloride

🛑
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7
Q

SEVERE lead toxicity = [(⬜serum lead level) or ⬜]

tx? (2)

A

≥70 (or encephalopathy)

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8
Q

Moderate lead toxicity = serum lead level of ⬜.

What’s unique about this level of toxicity ?

tx?

A

[Moderate lead toxicity = 45-69]

[XR lead lines from lead deposition on long bone metaphysis]

_________________

normal lead level = < 5

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9
Q

mild lead toxicity = serum lead level of ⬜

tx? (2)

A

5-44

tx = [no meds] and [repeat venous blood lead level in 1 month]
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

*normal lead level = \< 5*​

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10
Q

Lead inhibits ⬜ which causes what effect on RBC?

major features of lead toxicity -10

A

[ferrochelatase and ALAD] ; [⬇︎heme synthesis and ⇪ RBC protoporphyrin]

\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

**LL**\_**EE**\_**AA**\_**DD\_SS**

1-{**L**ead lines ([Burton's gingival lines] and on [metaphyses of long bones on xr]}

2-[A**L**AD and ferrochelatase] are inhibited by Lead

3-**E**ncephalopathy
4-**E**rythrocyte Basophilic Stippling (RBC retained rRNA aggregates due to Lead inhibiting rRNA degradation)
5-**A**bd colic
6-sideroblastic **A**nemia
7-**D**rops (wrist drop, foot drop, stocking glove)
8-[**D**imercaprol and EDTA are 1st line tx
9-***S**uc*cimer = peds tx ("it *Suc*ks to be a kid who ate lead")

10-**S**eventy-eight ⼀Houses older than 1978 have ⇪ for lead poisoning

## Footnote

*normal lead level < 5*

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11
Q

Iron deficiency is the most common pediatric nutritional deficiency and should be suspected in any child drinking greater than ⬜ cow’s milk /day

___________________

what is the order of physiological changes that occur after giving [ferrous sulfate] iron therapy?

A

>710 cc

_________________

[ferrous sulfate oral therapy] ➜ ([⇪ reticulocytes] ➜ [⇪ hct & hgb] ) by 1 mo

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12
Q

etx

Aplastic Crisis

A

🎃AC = [Aplastic Crisis)] = [ Anemia COMPOUNDED
_________________
🎃 Anemia of [SCD | HS] become an ANEMIC CRISIS if/when COMPUNDED with ParvoB19 infxn (since parvoB19 replicates in [proerythroblaSt_erythroid precursor]
_________________
🎃 … this is 2/2 parvoB19 replicates in (ProErythroblast_Erythroid precursors → [⬇reticulocyte RBC] → [SEVERE⬇︎Erythropoiesis= (⬇︎RBC/⬇︎Hgb)]
_________________
🎃
[SEVERE⬇︎Erythropoiesis= (⬇︎RBC/⬇︎Hgb)]2/2 ParvoB19

preexisting [⬇︎ O2 carrying capacity] from[S|HAnemia]

APLASTIC CRISIS

*{[anemia] from parvoB19} occurring in the setting of preexisting [S|HAnemia] ➜ “Crisis” because RBC reduction by ParvoB19 in setting of already- reduced RBC from [S|HAnemia] is… acute on chronic CRISIS!
___________________________x____________________________________
🔎[*S|H*Anemia] = {[Sickle Cell Disease_Anemia] and/or [Hereditary Spherocytosis_Anemia]}

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13
Q

What’s the difference between [Aplastic Crisis] and [Aplastic Anemia]?

A

AA = [(Aplastic Anemia)] =[All-cell PANcytopenia (including ⬇︎Erythropoiesis)] from [failure/destruction/suppression] of [Hematopoietic CD34 Myeloid Stem Cells] By [Bone Marrow T-cells( → hypOcellular bone marrow with fatty infiltration) = dry bone marrow tap] … These [Bone Marrow T-cells] are activated by [IDIOPATHIC > Myelotoxic Drugs/Body Radiation/Virus/Fanconi)]

_________________

AC = [AplasticCrisis)] = [Anemia in Sickle Cell pts,] 2/2 to parvoB19 replicating in (ProErythroblast_Erythroid precursors) → causes [⬇reticulocyte RBC] → [SEVERE ⬇︎Erythropoiesis (⬇︎RBC/⬇︎Hgb)] … in setting of already (sickle cell) anemic state = Crisis

[A C] from parvoB19 is worst in the presence of other anemias since RBC reserve will be reduced

Aplastic Anemia hypOcellular bone marrow
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14
Q

cp for Radiation proctitis (4)

A
  1. s/p pelvic radiation therapy
  2. tenesmus
  3. bloody diarrhea
  4. [anal mucus discharge]
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15
Q

ITP

tx for Adults? (4)

[Immune Thrombocytopenic Purpura (ITP)

A

-obs(cutaneous sx but platelet≥30k )

-[CTS | IVIG | antiD](if bleeding | platelet< 30k)

etx: Ab binds to platelet → both removed by Spleen macrophages → thrombocytopenia → purpura from uncontrolled bleeding

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16
Q

ITP

tx for peds? (4)

[Immune Thrombocytopenic Purpura (ITP)

A

-obs(if cutaneous sx only )

-[CTS | IVIG | antiD](if bleeding)

etx: Ab binds to platelet → both removed by Spleen macrophages → thrombocytopenia → purpura from uncontrolled bleeding

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17
Q

MethanOL overdose antidote

A

Fomepizole
_________________

inhibits [hepatic alcohol dehydrogenase] from converting EG/methanol ➜ nephrotoxic metabolites

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18
Q

[Ethylene Glycol (antifreeze)] overdose antidote

A

Fomepizole
_________________

inhibits [hepatic alcohol dehydrogenase] from converting EG/methanol ➜ nephrotoxic metabolites

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19
Q

In a hemorrhaging patient, when do you transfuse [pRBC]? (2)
_________________

pRBC = packed RBC

A

hgb< 7

(or < 8 if CVD/CA)

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20
Q

What are the early sx of Sickle Cell disease ? (2)
_________________

how is this diagnosed? ​

A

-dactylitis (painful swelling of hands/feet 2/2 bone infarction)

-hemolytic anemia
\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_

<sub>Electrophoresis</sub>: {[Hgb **S**​] with [NO Hgb **A**]}

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21
Q

Name the lab pair used to confrim SLE diagnosis

A

dana Smith

antidsDNA + anti-Smith

RASHH ORR PAINN
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22
Q

Hydoxychloroquine is a ⬜ used to treat what sx in SLE? (3)
_________________

How is CTS used in SLE patients? (2)​

A

anti-malarial ;

Rash / Arthralgia / Soft tissue synovitis ​
_________________

acute SLE = low dose CTS

Chronic/SEVERE SLE = HIGH dose CTS

RASHH ORR PAINN
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23
Q

RA is a common cause of Anemia of Chronic Disease

How do you treat ACD 2/2 RA?

A

Infliximab(AntiTNFα)

{ACD🔧: chronic/inflammation→ hepcidin→ [macrophage/intestinalferroportin]❌ from transfering Fe from FerriTin to Transferrin ➜ {[ Transferrin Saturation with paradoxic ⬇︎TIBC] , [ FerriTin],} and [⬇︎Fe**in circulation*],

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24
Q

Upregulators of Cytochrome P450 Enzymes (8)

A

Chronic alcoholics Steal Phen-Phen & Never Refuse Greasy Carbs which keeps them UP

  • Chronic alcohol use
  • St.John’s wort
  • Phenytoin
  • Phenobarbital
  • Nevirapine
  • Rifampin
  • Griseofulvin
  • Carbamazepine
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25
**Inhibitors** of Cytochrome P450 Enzymes (11)
**A**AA **RACKS I**n **GQ M**agazine *INHIBIT me from doing my job!* **A**cute Alcohol Abuse **R**itonavir (HIV Protease inhibitor) **A**miodarone [**C**imetidine & Ciprofloxacin] **K**etoconazole **S**ulfonamides [**I**NH Isoniazid] [**G**rapefruit Juice] **Q**uinidine [**M**acrolides *(except Azithro)*]
26
What **Substrates** does Cytochrome P450 metabolize? (6)
**C**an **A**lways **T**hink **W**hen **O**utdoors, **S**on...*.i need it!* ## Footnote [**C**yclosporine (Liver AND small intestine)] **A**ntiEpileptics **T**heophylline **W**arfarin **O**CP [**S**tatins (*NOT PRAVASTATIN*)]
27
*M.D. must recognize early signs of [Phenytoin toxicity \> ⬜ mcg/ml]* What's the earliest sign?
20 [Nystagmus on far lateral gaze] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *other signs: [diplopia, ataxia ➜ coma]* [Phenytoin 10-(therapeutic)-**20** \< (TOXIC)]
28
Tx for Clostridium Botulinum poisoning - 3
1. [Equine Heptavalent **Anti- _Botulinum_ *_toxin_*** (passive immunity)] 2. [Ig **Anti- _Botulinum_**] 3. Guanidine
29
Jimson Weed Poisoning clinical presentation - 7
*Jimson Weed = AntiCholinergic* "Blind as a bat, mad as a hatter, red as a beet, hot as a hare, dry as a bone, the bowel & bladder lose their tone, and the heart runs alone....." 1. Blind as a bat = [**Mydriasis and [cycloplegia** (blurry vision especially when focusing on near objects)] 2. Mad as a hatter= **Agitation & Hallucinations** 3. Red as a Beet = **Cutaneous flushing** despite vasoconstriction 4. Hot as a hare = **Hyperthermia** from DEC ability to sweat 5. Dry as a bone= **DEC Secretions** (including sweat) 6. **Bladder & Bowel lose tone** 7. Heart runs alone = No vagal tone at SA --\> **Tachycardia**
30
[MMalignant Hyperthermia] etx
After giving [**inhaled anesthestics** vs **succinylcholine**] to genetically predisposed (AUTO DOM) pts develop ➜ ## Footnote **M**alignant = **M**uscle Rigidity **Malignant** = Malignant Unstable Vitals **Hyperthermia** = Fever
31
MMalignant Hyperthermia Tx
Dantrolene ## Footnote *TREAT PROMPTLY! AS THIS IS LIFE THREATENING CONDITION!*
32
TTP treatment​ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *TTP = Thrombotic Thrombocytopenic Purpura*
plasma **EXCHANGE** \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *(Tx = _EXCHANGE_ plasma containing anti-A13vM or deficiency of A13vM for normal plasma)* | A13vM= [ADAMTS-13-vWF Metalloprotease]) ## Footnote TTP etx: [A13vM❌(or inhibition by shiga toxin in HUS)] →uncontrolled vWF chain cleavage → widespread platelet traps/activation → thrombocytopenia + microagio hemolytic anemia → _FMNRT_ TTP cp ⚠️TTP cp (aside from +Neuro and plasma EXCHANGE tx) = HUS cp. ⼀ *(TTP Tx = _EXCHANGE_ plasma containing anti-A13vM or deficiency of A13vM for normal plasma)*
33
TTP MOD ​(4) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *TTP = Thrombotic Thrombocytopenic Purpura*
① *{[**A13vM** (ADAM**T**S13\_vWF Metalloprotease)] (responsible for degrading vWF Multimers)}* ② A13vM❌ = [acquired A13vM inhibition(by shiga toxin in HUS)] or [ acquired A13vM deficiency] *(2/2 CLOpidogrel | tiCLOpidine | cyCLOsporine | AIDS )* ③ ➜ allows ⇪ vWF Multimers to circulate and start cleaving large chains of von Willebrand factor from the vascular endothelium ➜ *cleaved* vWF ➜ widespread platelet traps and platelet activation ➜severe thrombocytopenia ④ +{RBC shearing ➜ [Schistocyte helmet cells], [INC Direct bilirubin], [microangiopathic hemolytic anemia]} * * * ☞ TTP Symptom PENTAD (**FMNRT)** \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *tx = plasma **EXCHANGE** {(exchanges [anti-A13vM] and/or [low A13vM])for [more A13vM])}* ## Footnote *💡in HUS, shiga toxin inhibits A13vM → similar TTP (but w/out neuro sx or plasma EXCHANGE tx) clinical presentation*
34
In RBC... Adult Hemoglobin is made of 4 globin chains. What are they? ​fetal Hemoglobin? ​ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ βeta Thalassemia occurs because of ⬜ on Chromosome ⬜ of either parent. This → β-chain being ⬜ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ describe the 2 variants of βeta thalassemia *βeta Thalassemia = SEVERE anemia*
hgbA_ADULT[αα⼀αα / β⼀β] ​ hgbF_fetal[αα⼀αα / γ⼀γ] ​​ * * * [point mutations in splice & promoter sequences]; 11; [underproduced or ABSENT] * * * [αα⼀αα / ❌⼀β] ​ = **βTminor **(➜ β-chain underproduced= *asx, [⇪ HgbA2= dx],* ) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [αα⼀αα / ❌⼀❌] ​ = **βTMAJOR **(➜ β-chain ABSENT = *early death , ⇪ HgbF|| temp tx = Hypertransfusion*) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_​ * \*αα (2 genes) = 1 α globin chain\* | βT = βeta Thalassemia* * causes microcytic hypOchromic anemia*
35
In RBC... [*Adult* Hemoglobin A] is made of 4 globin chains. What are they? ​fetal Hemoglobin? ​ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ αlpha thalassemia occurs due to ⬜ on chromosome ⬜. This can ultimately → 5 variants of hemoglobin makeup Describe all 5 αT variants *alpha Thalassemia*a
hgbA_ADULT[αα⼀αα / β⼀β] ​ hgbF_fetal[αα⼀αα / γ⼀γ] ​​ [αlpha allele deletion (2 alleles per parent);] chromo 16 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ I:[⬜α⼀αα / β⼀β] ➜ [**αT**⼀silent carrier] * * * II:[⬜α⼀⬜α / β⼀β] ➜ [**αT**⼀***trait*****TRANS**] II:[⬜⬜⼀αα / β⼀β] ➜ [**αT**⼀***trait*****CIS(worst for offspring)**] * * * III:[⬜⬜⼀⬜α / β⼀β] ➜ [**αT**⼀**hb**] * * * IV:[⬜⬜⼀⬜⬜/ β⼀β] ➜ [**αT**⼀**🅱🅶**] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [**αT**⼀**hb**] = Hb(h)****tetramer of beta]** [**αT**⼀**🅱🅶**]= {[h🅱:T🅶 :HFD]**} = {Hgb**** 🅱ART tetramer of 🅶amma (Hydrops Fetalis(DIES IN UTERO))**]} ​ *\*αα (2 genes) = 1 α globin chain\* | αT = alpha Thalassemia*
36
[Microcytic hypOchromic anemia] likely indicates the congenital hemolytic anemia ⬜. Why are these patients at risk for organ damage 2/2 iron overload ? (3)
[*Beta* Thalassemia MAJOR [αα⼀αα / ❌⼀❌]] ; Hypertransfusion tx overcomes effects of anemia and extramedullary hematopoiesis **BUT** also ➜ iron overload ➜ severe organ damage from iron deposition
37
[Anemia with *Normal* RDW] typically indicates \_\_\_\_
Thalassemia
38
What is Cooley Anemia? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ tx?
Beta thalassemia MAJOR (BOTH Mom and Dad [chromo 11 β-globin genes] have [splice/promoter point mutations] →2 out of 2 ABSENT β-globin → [⇪ HgbF (a2g2)] = SEVERE COOLEY ANEMIA \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Tx = [chronic blood transfusion **with deferadirox iron chelator**]
39
DDx - 4
Target cells = **HALT** 1. **H**bC 2. **A**splenia 3. **L**iver disease 4. **T**halassemia (usually asx and REQUIRES NO TX if asx - occurs in Mediterranean people)
40
Dx
Thalassemia ## Footnote *image shows teardrop cells (Thalassemia also has Target cells)*
41
What must you be VERY CAUTIOUS of when treating Megaloblastic macrocytic anemia with Vitamin B12? Explain
hypOkalemia! (within 48h of VB12 tx) ​ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ VB12 supplement in moderate/Severe Megaloblastic macrocytic anemia will ➜ newly formed RBC ➜ **RAPID INTRACELL UPTAKE OF K+ by new RBC** ➜ hypOkalemia! So... [transfuse pRBC before VB12 ➜ blunts new RBC synthesis ➜ prevents hypOkalemia] + [monitor K+ 48h]
42
clinical presentation of neonatal polycythemia (5)
1. neonatal [**PERIPHERAL** venous hct \> 65%] 2. \*\*[ASX v 24h self limited] \*\* 3. [+/- life threatening apnea] 4. [+/- ​hypOglycemia] 5. [+/- hyperviscosity (*causes hypOperfusion ➜ lethargy, hypOtonia*)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *1st hct comes from heel prick but = unreliable / confirmed by peripheral venous if abnml*
43
treatment for neonatal polycythemia (3)
1. [**HYDRATION]** **2. CORRECT METABOLIC DERANGEMENT** 3. --(if persist)--\> [Partial exchange transfusion]
44
*Patients with Multiple Myeloma need skeletal system assay for bone involvement* How is this done?
[Whole-Body-Cross-Sectional] eval with **[low-dose CT no contrast]** \> MRI or PET
45
What is hyperviscosity syndrome?
excessive production of **monoclonal IgM** (Waldenstrom Macroglobulinemia \> Multiple Myeloma) ➜ congestion of brain's microcirculation➜ [neuro ∆ /hearing ∆ /vision ∆] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ neuro ∆ = somnolence/coma/HA​ ## Footnote *tx = plasmapheresis*
46
There are many causes of [*Transient* elevated PSA], in which PSA should normalize by ⬜ *but* What are the only 3 causes of [**PERSISTENT** elevated PSA]?
4-6 wks; ## Footnote 1. [CP/CPPS] 2. BPH 3. Prostate CA \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_x\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *(Chronic Prostatitis⼀Chronic Pelvic Pain Syndrome)*
47
What do patients use Ginkgo biloba for? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What's the major potential side effect?
["memory booster" (2/2 to its suggested propensity for ⇪ cerebral blood flow)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Bleeding(*inhibits platelet-activating factor + potentiates anticoagulants*) (especially if combined with ASA/antiplatelet drugs) | *"[herbs **GSG**] cause Bleeds"*
48
Explain the relationship between [Tumor Lysis Syndrome] and Cardiac arrest (6)
Chemotherapy commonly ➜ {TLS = [⇪PU**K**ED (and ⬇︎Ca+)]} --(if SEVERE hyperKalemia)--\> [sine wave Wide QRS] ➜ VENTRICULAR ASYSTOLE ➜ CARDIAC ARREST *PUK = Phosphate/Uric acid/K+*
49
FFP is used for what purpose? (2)
1. DIC 2. *2nd line tx for*[Vitamin K deficiency coagulopathy(i.e. warfarin OD INR ≥2)] ; ## Footnote *(2nd line tx for Warfarin OD because FFP requires large volume > 2L)*
50
When is Platelet transfusion indicated?
[**platelet < 50K**]
51
Laboratory findings for [Cobalamin VB12] deficiency (3)
* **PAN**cytopenia * Macrocytic anemia * low reticulocyte count ## Footnote *common cause of delirium/dementia in elderly!*
52
Vegan Elderly patients p/w delirium or dementia should always make you c/f ⬜
[Cobalamin B12] deficiency!
53
Describe the 2 molecular reactions [Cobalamin B12] is responsible for
54
How does [Cobalamin VitB12] deficiency lead to hyperbilirubinemia
[Cobalamin VitB12 deficiency] ➜ defective DNA synthesis in bone marrow ➜ [Erythroid hyperplasia but with no maturation] = **RBC megaloblastic transformation** ➜ [intramedullary RBC hemolysis] ➜ [⇪ hemolysis markers ⼀indirect bilirubinemia, LDH, (low haptoglobin)] and no reticulocyte response
55
how do you emergently reverse Warfarin OD? (2)
**[PCC +KIV]** \> ffp \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ * PCC = Prothrombin Concentrate Complex (contain VitK factors and normalizes INR\<10m) / KIV = Vitamin K IV(takes 12h to onset)* * ffp= fresh frozen plasma(2nd line due to large volume \>2L required)*
56
These 3 agents emergently reverse ⬜ Briefly Describe each a. [PCC ⼀*ProThrombin Complex Concentrate*] b. [KIV ⼀*Vitamin K IV*] c. [FFP ⼀*Fresh Frozen Plasma*]
**WARFARIN** **[PCC +KIV]** \> ffp \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ a. PCC = contain [VitK factors2/7/9/10] and normalizes INR\<10m b. KIV = takes 12h to onset c. ffp = 2nd line for warfarin reversal due to [large volume \>2L required]
57
# Warfarin MOA
58
# Treatment for Warfarin toxicity depends on INR and Bleeding *_Name the [Warfarin toxicity] tx*_ (2) _*for:_* [*(supratherapeutic) *INR: 3-4.5] with [Bleeding: ≤minimal]
[Hold Warfarin x 1-2d] | [DEC dose]
59
# Treatment for Warfarin toxicity depends on Bleeding and INR *_Name the [Warfarin toxicity] tx*_ (2) _*for:_* [Bleeding: ≤minimal] with [*(supratherapeutic) *INR: 4.5-10]
- Hold Warfarin ➜ Resume when INR therapeutic - [vitKLd 1- 2.5mg PO]*(If INC risk of bleeding)** * \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *[vitKLd PO]: vitamin K oral (Low dose = 1-2.5mg)*
60
# Treatment for Warfarin toxicity depends on INR and Bleeding *_Name the [Warfarin toxicity] tx*_ (2) _*for:_* [*(supratherapeutic) *INR: \>10] with [Bleeding: ≤minimal]
- Hold Warfarin ➜ Resume when INR therapeutic - ***GIVE*** [vitK**HD** 2.5 - 5 mg PO] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *[vitKHD PO]: vitamin K oral (HIGH Dose =2.5 -5mg)*
61
# Treatment for Warfarin toxicity depends on INR and Bleeding *_Name the [Warfarin toxicity] tx*_ (3) _*for:_* [INR: x] with [Bleeding: **SEVERE**]
- Hold Warfarin ➜ Resume when INR therapeutic - ***GIVE*** [vitK**UHD**10 mg IV] - ***GIVE*** [PCC] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *[vitKUHD IV]: vitamin K IV(ULTRA HIGH Dose = 10mg)*
62
What is Pernicious Anemia? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ how is this diagnosed?
[**Anti-ParietalCell** and **Anti-IntrinsicFactor**] autoimmune destruction ➜ AMAG ➜ no IntrinsicFactor to facilitate {[Cobalamin VB12] terminal iLeum absorption} ➜ [mAcrocytic megaloblastic anemia] dx = elevated Anti-IF | AMAG = Autoimmune Metaplastic Atrophic Gastritis
63
# \*HIGH YIELD\* Name the gastritis associated with pernicious anemia \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What are the 3 main components of it's MOD
AMAG = autoimmune destruction against intrinsic factor and oxyntic cells ➜ gastric **fundus** and gastric **body** destruction with: 1. glandular atrophy 2. intestinal metaplasia 3. intestinal inflammation \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *AMAG = Autoimmune Metaplastic Atrophic Gastritis*
64
What is Tumor Lysis Syndrome? (5)
{[cytotoxic chemotherapy] or [high grade lymphoma]} ➜ rapid lysis of neoplastic cells ➜"*pt* **PUKED**" - **P**hosphate INC (⇪ [serum/urine *P*] binds [serum/urine *Ca+*] ➜ [⬇︎serum *Ca+* and CaPhosphate renal stones]) - {**U**ric acid INC from pUrine DNA bases (serum and [urine ➜ Uric Acid stones]) = (px=allopurinol and IVF)} = DIAGNOSIS - **K**+ INC ( ➜ arrhythmia) - [**E**levated (Ca+P & Uric Acid)renal stones ➜ AKI (IVF px)] - [**D**NA base *Purines*] INC → Uric acid INC
65
# Rasburicase MOA?
[urate oxidase*(not naturally in humans)* recombinant] = catalyzes conversion of [INC Uric acid(i.e. from Tumor Lysis Syndrome)] ➜ [allantoin (excreted in urine)] for Tumor Lysis Syndrome px \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
66
# Cancer pt on Rasburicase presents with AKI after starting chemo Why is this? Why didn't Rasburicase stop it?
* Cancer = chemo ➜ [TLS **PUKED**] ➜ [**E**levated (Ca+P & Uric Acid)renal stones ➜ AKI] * Rasburicase only ⬇︎Uric Acid renal Stones. AKI can still be caused by Ca+P stones
67
# Febuxostat MOA
68
# Allopurinol MOA
69
Why is ChemoRadiation Therapy given together for Head/Neck CA versus Chemo or Radiation alone?
[60% Head/Neck CA are locally advanced at time of dx = inoperable] ➜ CRT as a duo INC the 5 year survival rate
70
*Violent* PCP should be treated immediately with ⬜ ⼀and *Mild* PCP should be treated with ⬜2
*Violent* PCP = [**BENZO** sedation] *Mild* PCP = [low stimulation environment +/- benzo sedation] *** *[PCP (Phencyclidine)] is a [NMDA R Blocker hallucinogen]*
71
How do you manage a patient using herbal medicine against medical advice (4)
* physician should* 1. explain risks of herbal preparations 2. **document** that you counseled patient to avoid herbal prep 3. **document** patient refusal/response to avoid herbal prep 4. follow patient closely for adverse effects
72
List the differences of [*Peripherally* Inserted central catheters (PICC)] as compared to [*Centrally* Inserted central catheters] (4)
PICC has [**HIGHER** UE DVT] but… PICC has **lower** - infections - procedural complications - patient discomfort
73
Describe the following values for Iron Deficiency Anemia: MCV Iron [Transferrin saturation] TIBC Ferritin
74
What is TIBC
*Total Iron Binding Capacity = TIBC = "**T**ransferrin **I**n **B**lood **C**alculation"* = [**TOTAL amount of transferrin***(transports iron)* **in serum**] (by calculation)
75
Describe the following values for Thalassemia: MCV Iron [Transferrin saturation] TIBC Ferritin
76
Describe the following values for Anemia of Chronic Disease: MCV Iron [Transferrin saturation] TIBC Ferritin
## Footnote {ACD🔧: chronic/inflammation→IL6 secretion → [hepatic hepcidin secretion]→ [macrophage/intestinalferroportin]❌ from *transfering* Fe from FerriTin to Transferrin ➜ {[ **↧** Transferrin Saturation **with an accompanying** Transferrin "activity pause" *= stops Transferrin from scavenging blood for more Fe* → ⬇︎ **T**ransferrin **I**n **B**lood (⬇︎TIBC)] , [**↥** FerriTin],} and ultimately [⬇︎Fe**in circulation*],
77
Describe the following values for Sideroblastic Anemia: MCV Iron
⬇︎MCV ⬆︎Iron
78
[**4-allele deletion**Alpha thalassemias] are characterized as ⬜ on electrophoresis
[HgbBarts tetramer of gamma (Hydrops Fetalis)] | *Intrauterine death 2/2 CHF*
79
What is [Hemoglobin SC] disease? (3)
- less severe variant of [Sickle Cell Disease (Hgb_SS & Hgb_SC)] - ➜ mild normocytic anemia - electrophoresis = [equal Hgb\_S = Hgb\_C]
80
Traditionally, FerriTin less than ⬜ indicates Iron Deficiency, but realistically why can this not be a hard rule?
[FerriTin \< 15] = IDA ## Footnote \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Most pts with [FerriTin 15-30] realistically are also iron deficient = Traditional rule can only **rule IN IDA**
81
Multiple Myeloma tx - 2
1. [BorTezomib proteasome inhibitor] OR 2.{[LD *and if \<70 yo*(➜ BMT)} *** ## Footnote *[Lenalidomide + Dexamethoasone] --\> [Bone Marrow Transplant]}*
82
What is the issue with seeing IgG or IgA monoclonal "M" spikes on serum protein electrophoresis?
This does **NOT** automatically mean Multiple Myeloma. This can be seen in MGUS-Monoclonal Gammopathy of Unknown Significance which = common in older pts and only transforms to Multiple Myeloma 1%/year
83
Waldenstrom Macroglobulinemia MOD \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ cp-5
Multiple Myeloma plasma cells overproduce Ig**M** specifically --\> hyperviscosity sx (HA, tinnitus) 1. Neuropathy 2. Engorged blood vessels 3. HA 4. tinnitus 5. Raynaud Phenomenon
84
Waldenstrom Macroglobulinemia tx - 3
1. **PLASMAPHERESIS initially** 2. Chlorambucil + Prednisone = long term OR 3. Fludarabine + Prednisone = long term
85
tx for Von Willebrand disease - 3
DDAVP desmopressin --(refractory)--\> Factor 8 or vWF concentrate ## Footnote *DDAVP → ⇪Factor 8 → ⇪ vWF* (DDAVP releases subendothelial stores of vWF)
86
abx choice for [Acute Chest Syndrome in Sickle Cell Disease] -2
CefTriaxone (*Strep Pneumo*) + Azithromycin (*Mycoplasma PNA*)
87
treatment for iron overload in Hemochromatosis
therapeutic phlebotomy
88
Which CA presents as a pathologic lymph node of the head & neck in a smoker/EtOH patient?
metastatic SQC
89
# MELAS stands for ⬜ Describe the clinical features (3)
**MELAS** [**M**itochondrial **E**ncephalopathy + **L**actic **A**cidosis + **S**troke-like episodes] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 1. stroke-like episodes, lactic acidosis, seizures, m weakness, hearing loss 2. onset LOE40 yo 3. maternally transmitted only
90
SLE patients are at INC risk for developing which CA?
[Non-Hodgkin Lymphoma: (DLBL)] *Diffuse Large B-Cell* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ ***RAS_HH_ O_RR_ PAI_NN_***
91
Clinical findings of Frostbite (6)
*"Frostbite **numbs** your body, **scars** your body, then **Kills**your body"* ## Footnote 1st: **numbs** = [(Pallor +/- Thrombosis) + Anesthesia] 2nd: **scars**= [blister + eschar] 3rd: **Kills** = [Deep Tissue Necrosis, Mummification]
92
What is the treatment for Frostbite? (5)
93
# In Frostbite, Thrombolysis is a treatment option Initial treatment for Frostbite is ⬜. When is Thrombolysis considered to be used? (3)
[Rapid rewarming in water bath at 37-39C] ; * * * *Thrombolysis is considered in patients who:* 1. have absent perfusion on angiography or technetium-99m scan 2. amputation would be suboptimal 3. seek care within 24h of injury *(typically only used in these pts)*
94
# Malaria Clinical features? (4)
1. [FEVER + HA + THROMBOCYTOPENIA] in travelers returning from sub-Saharan Africa 2. Dx = peripheral blood smear 3. Plasmodium falciparum 4. [Antimalarial prophylaxis before, during and after]
95
*Although this herbal preparation has no clinically proven efficacy,* List the conditions it's associated with treating: * * * **Saw Palmetto**
BPH | *"GSG cause Bleeds"*
96
*Although this herbal preparation has no clinically proven efficacy* List the conditions it's associated with treating: * * * **garliC**
Hyper**C**holesterolemia | *"[herbs **GSG**] cause Bleeds"*
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* Although this herbal preparation has no clinically proven efficacy,* * List the conditions it's associated with treating:* * * * **glucosamine**
Osteoarthritis
98
* Although this herbal preparation has no clinically proven efficacy,* * List the conditions it's associated with treating:* * * * **chondroitin**
Osteoarthritis
99
* Although this herbal preparation has no clinically proven efficacy,* * List the conditions it's associated with treating:* * * * **St.John's wort**
Depression ## Footnote **Chronic alcohol**ics **St**eal **Phen-Phen** & **N**ever **R**efuse **Gr**easy **Carb**s *which keeps them UP*
100
List the herbal preparations that cause bleeding (3)
*"[herbs **GSG**] ... Bleeds"* ## Footnote -**G**inkgo biloba -**S**aw Palmetto -**G**arlic
101
CYanide is released during the combustion of items containing the compounds [⬜ and ⬜]. Give 2 examples of materials that contain both these compounds
Carbon & Nitrogen ; PLASTIC | Polyurethane foam ## Footnote *{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}*
102
Describe why patients with CYanide poisoning have [*bright CHERRY**Red** skin*]
[CYanide(*released from burning PLASTIC|using NitroprussideRx*)] inhibits [mitochondrial oxidative phosphorylation] ➜ forces cell to switch to anaerobic metabolism ➜ lactic acid formation = [lactic acidmetabolic acidosis]. unused arterial oxygen remains in blood ➜ unused venous oxygen = [*bright CHERRY**Red** skin*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ tx = [Hydroxocobalamin +/- Sodium thiosulfate] ## Footnote *{CYanide tox sx = [*bright _CHERRY_**Red** skin*], Yucky bitter almond breath, brain⇪*seizure*, brain⬇︎*CNS depression* , [lung⇪ f/b lung⬇︎*]*[tachypnea f/b bradypnea]*
103
*Both CYanide and Carbon MONOxide poisoning cause bright Red Skin* How can you differentiate the two? (2)
1. [CYanide (*released from burning PLASTIC*)] inhibits [mitochondrial oxidative phosphorylation] vs [CO binds hgb with higher affinity than O2 ➜ DEC oxygen delivery] 2. [CO = Carboxyhemoglobin \> 25%] vs [CYanide = nml Carboxyhemoglobin] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ ## Footnote *{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}*
104
Sx of CYanide poisoning (6)
1. Bright CHERRY Red Skin (*also seen in CO poisoning*) 2. Yucky Bitter Almond breath 3. brain⇪*seizure* 4. brain⬇︎*CNS depression* 5. [lung⇪ f/b lung⬇︎]*[tachypnea ➜ bradypnea]* 6. [lactic metabolic acidosis] | *🧠inhibits oxidative phosphorylation → anaerobic metabolism → lacticMA* ## Footnote *{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}*
105
Why do pts receiving Nitroprusside have INC risk for ⬜ toxicity? (2)
CYanide; ▶Nitroprusside (rapid short acting vasoDilator) metabolizes into both: - Nitric Oxide ➜ vasoDilation - **CYanide ➜ converted to thioCYanate ➜ RENAL excreted** \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ ▶Patients with renal insufficiency = unable to excrete thioCYanate ➜ CYanide toxicity ## Footnote *{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}*
106
What's the most common inherited thrombophilia?
Factor 5 Leiden
107
Describe the order of events for ordering blood transfusion for a patient (4)
*blood is ordered for transfusion:* **T**ype **S**erum **C**arefully, **D**ude 1st: **Ty**pe: [Recipient patient's] blood is *typed* = ABO and Rh determined \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 2nd: [**S**creen (PTAS)]: Recipient's serum **_S_**creened for [unexpected *preexisting* autoantibodies (*previously made* against RBC antigens E, L or K from previous transfusions)] = [**_P_**re**_T_**ransfusion **_A_**b **_S_**creen] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 3rd: **C**rossmatch Recipient's blood with [blood they will be receiving] in-vitro first \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 4th: **D**ecide: [**✅Transfuse (***⊕unexpected e/l/kRBC AutoAntibodies or ⊕Crossmatch rejection?***)🚫Transfuse ➜ Identify Ab**]
108
[GVHD-Graft Versus Host Disease] is common after _____ or _____ transplant \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ It involves [Graft\_Donor ⬜ cells] attacking which 3 parts of the [Host_Receipient body]?
Bone marrow; Organ ; \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ T ; 1. Skin 2. Liver 3. GI *This occurs because [Graft\_Donor] T-cells recognize major and minor HLA antigens of the [Host_Recipient Skin//Liver/GI]*
109
What is the px for [Febrile nonhemolytic transfusion reaction]
Leukoreduction of [graft\_donor] blood \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *this will also ⬇︎ risk of HLA alloimmunization and CMV transmission*
110
What is Leukoreduction used for? (3)
*[Leukoreduction of [graft\_donor] blood] will ⬇︎ risk of:* 1.**[Febrile nonhemolytic transfusion reaction]**, 2.[HLA alloimmunization]✳ 3.CMV transmission*(CMV resides in Leukocytes)* ## Footnote ✳alloimmunization: = *host_recipient immune response to otherwise [foreign antigens (blood group ag|histocompatibility ag) from ANOTHER PERSON] [⼀usually from incidental exposure like transfusion] *
111
A patient receiving Nitroprusside for Aortic Dissection develops acute metabolic acidosis and confusion. Diagnosis?
Cyanide toxicity ## Footnote *{{CYanide is released from [burning PLASTIC] or [NitroprussideRx]} | Tx = Hydroxocobalamin+/- Sodium thiosulfate}*
112
# The 3 phases of [Primary Hemostasis platelet plugging] are \_\_\_\_ Describe the 3 phases of [Primary Hemostasis platelet plugging *(1HS)*] that *stops the bleeding after you cut your arm*
“superficial cut using **_DAG_** on it !” 1st: [a**D**herence] *of circulating [inactivated platelet's Gp1B] to exposed vWF of subendothelial collagen* →2nd: [a**A**ctivation & secretion] = *Activated platelet → secretes fibrinogen and {[ThromboxaneA2 & ADP] → [INC (Gp2b/3a\_platelet fibrinogen R) expression on platelets]}* *→* 3rd: [a**G**gregation] *of platelets via fibrinogen binding them together via [Gp2b/3a\_platelet fibrinogen R]* = [*weak* 1*º* PLATELET PLUG] *\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_*
113
How does Chronic Kidney Disease cause [mucosal bleeding1HS❌]? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *1HS❌: [Primary Hemostasis platelet plugging _disruption_ ]*
114
patients with [Chronic Kidney Disease] have INC ____ dysfunction → what clinical presentation? (5)
platelet; * CKD patient * [1HS❌ *(⇪ **mucosal bleeding/bruising**)*] * [platelet count nl] * [PT/INR nl] * [aPTT nl] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *1HS: [Primary Hemostasis platelet plugging]*
115
a. [Chronic Kidney Disease] causes dysfunction of [\_\_\_\_ cells] which → \_\_\_\_\_\_\_\_ b. How does this happen?
a. platelet; [1HS❌ *(⇪ **mucosal bleeding/bruising**)*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *1HS: [Primary Hemostasis platelet plugging]*
116
a. [Chronic Kidney Disease] causes dysfunction of [\_\_\_\_ cells] which → \_\_\_\_\_\_\_\_ * * * c. Treatment? (2)
a. platelet; [1HS❌ *(⇪ **mucosal bleeding/bruising**)*] * * * c. 1. Sx = [desmopressin dDAVP *(INC vWF secretion from endothelial cell)*]IV or SQ 2. Asx = observe \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *1HS: [Primary Hemostasis platelet plugging]*
117
a. [Gp2b/3A receptor] is found on the ___ cell, is called ___ when deficient, and is responsible for what? * * * b. Name the [Gp2b/3A R Blockers] (3)
a. platelet; [glanzmann thrombasthenia] ; a**G**gregates platelets (---by binding [*Activated* platelet Gp2b/3A receptor] with a another [*Activated* platelet Gp2b/3A receptor] via fibrinogen) * * * b. “he **ATE** my Gp2b/3A”: [**A**bciximab, **T**irofiban, **E**ptifibatide] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
118
[Gp1b] is a protein found on the ⬜ cell --- It is called ⬜ when deficient,-- and is responsible for what? ## Footnote * * *
a. platelet *[inactivated circulating platelets]*; [Bernard-Soulier syndrome] ; [[inactivated circulating platelets] a**D**heres to [vWF*Gp1b Receptor* *(since vWF will already be bound to the exposed subendothelial collagen)* ## Footnote →platelet [**A**ctivation & secretion*(of "F.A.T." stuff)*] → platelet a**G**gregation = [**DAG** 1HS]
119
In addition to analgesia, why is ASA also considered a blood thinner? (2)
ASA inhibits ***COX*** * * * ✏️ { [platelet Arachidonic Acid] ---(via ***COX***)-→ converted to [TXA2 (thromboxane)] → [platelet TXA2 (ADP also)] INC expression of [Gp2b/3A\_platelet fibrinogen R] which enables→ [platelet aGgregation] } ✏️ASA inhibits ***COX*** → No [TXA2 (thromboxane)]= No platelet aGgregation = No Clotting ("thins" your blood)
120
Which bleeding reversal agent should be used to treat DIC?
ffp
121
# DVTs can either be primary or secondary Describe what the main differences of secondary DVT are (4)
1. reversible or 2. time limited 3. (*ex:* surgery, pregnancy, OCP, trauma) 4. _[Warfarin tx for_ **_only_** _3-6 months_ *_(_ ≥6 mo for [1° idiopathic])*]
122
What is the symptom triad for acute opioid intoxication
* depressed brain,* *[= Somnolent/AMS]* * depressed blacks,* *["-pupils" = miosis])* * depressed breath* *[= shallow bradypnea ≤12 RR]*
123
# Organophosphates MOA = ⬜ Where are they found? (2)
[potent inhibitors of ACh**E**] → [No ACh*--breakdown*] → [ACh accumulation] → hyperactivation of *mCN* receptors = {[“*OTC* **dUMBBELLS C**reate **M**uscle”] ? …….**PAID**} * * * [agricultural pesticides], [sarin(WARFARE AGENT)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *mCN: muscarinic | CNS | Nicotinic*
124
# Organophosphates are potent inhibitors of ⬜ which leads to ⬜ Sx (11)
ACh**E** ; [ACh accumulation] → {[“*OTC* **dUMBBELLS C**reate **M**uscle”] ? …….**PAID**} * * * [“*OTC* **dUMBBELLS C**reate **M**uscle”] *_O_*rganophosphate ***_T_**oxic* *_C_*holinergic : 1. [**_d_**iarrhea/**U**rination/**M**iosis/**B**radycardia/**B**ronchospasm( → Respiratory failure) /**E**mesis/**L**iquid lung bronchorrhea /**L**acrimation/**S**alivation]*muscarinic* 2. [**_C_**oma/Seizure]*CNS* 3. [**_M_**uscle(weakness/paralysis/fasciculation)]*Nicotinic*
125
# Organophosphates are potent inhibitors of ⬜ which leads to ⬜ Treatment (4)
ACh**E** ; [ACh accumulation] → {[“*OTC* **dUMBBELLS C**reate **M**uscle”] ? …….**PAID**} * * * **PAID** 1. [**P**ralidoxime ⼀*reactivates ACh**E***] 2. [**A**tropine anticholinergic⼀ *competitively inhibits ACh*] 3. [**I**ntubation prn + ABCs (*give activated charcoal if exposure ≤1h)*] 4. [**D**econtamination]
126
cp for [Niacin B3] deficiency (4)
**DDDD***(AKA Pellagra)* **D**ementia [**D**ry mouthSTOMATITIS / CHEILOSIS] **D**iarrhea [**D**ermatitissymmetrical/blister vesicles in sunexposed areas] | *💡Niacin uses _Tryptophan_ for synthesis, & CaRcinoid tumors do also*
127
Radionuclide bone scans detect ⬜, which makes it sensitive for detecting [**⬜** osteoLytic | osteoBLASTIC] bone metastasis (*from ⬜* )
areas of INC bone turnover; osteo**BLASTIC***(prostate/SOLC/Hodgkin)*
128
Name the Cancers that metastasize into [osteoBLASTIC bone lesions] (3) * * * *Pt with osteoBLASTIC CA c/o dull shoulder pain, worse at night* How do you work them up for potential bone metastasis? (4)
Prostate / SOLC / Hodgkin * * *
129
Name the Cancers that metastasize into [osteoLytic bone lesions] (3) * * * * Pt with osteoLytic CA c/o dull shoulder pain, worse at night* How do you work them up for potential bone metastasis? (3)
* nMn* * N*onSOLC / *M*ultiple Myeloma / *N*onHodgkin * * *
130
Name the Cancer that metastasizes to form [mixedosteoBLASTIC or osteoLytic bone lesions] * * * * Pt with mixed osteoB/L CA c/o dull shoulder pain, worse at night* How do you work them up for potential bone metastasis? (5)
BREAST * * *
131
Tx for Sickle Cell Pain Crisis - 4
1.Dehydration = [**IV Hydration + IV Analgesia**] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 2.[Infxn (⊕fever|⊕WBC)] =**cefTriaxone** \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 3.Cold🌡=**[Transfusion*(if ⊕CVA|Retinal infarct|aCS| Priapism)*** \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 4."Kan't breathe_hypoxia" = **[O2 + Hydoxyurea *to carry more O2**⇪HbF*]** | *treat the 4 SCD triggers (DICK)* ## Footnote *aCS = acute Chest Syndrome*
132
Tx for Hereditary Spherocytosis - 2
1. [Folic acid B9] chronically 2. Splenectomy (stops hemolysis)
133
When is it ok to give Platelet transfusion to HUS|TTP?
**NEVER!!!** | *give plasma EXCHANGETTP tx|supportiveHUS tx* ## Footnote TTP etx: [A13vM❌(or inhibition by shiga toxin in HUS)] →uncontrolled vWF chain cleavage → widespread platelet traps/activation → thrombocytopenia + microagio hemolytic anemia → _FMNRT_ TTP cp ⚠️TTP cp (aside from +Neuro and plasma EXCHANGE tx) = HUS cp. ⼀ *(TTP Tx = _EXCHANGE_ plasma containing anti-A13vM or deficiency of A13vM for normal plasma)*
134
What is the most common adverse reaction to blood transfusion? * * * What causes it? * * * When does it present?
*1-6h*[**Febrile Nonhemolytic** **transfusion reaction**] = F/C from cytokine accumulation during blood storage]
135
Autoimmune Hemolytic Anemia and Hereditary Spherocytosis BOTH can cause extravascular hemolytic anemia How can you discern the two? - 2
[*DAT*] and [fam hx] * * * AIHA = [**⊕***DAT*] (negative fam hx) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Hereditary Spherocytosis = [⊝*DAT*] (positive fam hx) * * * * DAT* = [*_Direct_*(Coombs) *AntiHumanGlobulin Test*]
136
What is the purpose of the [**I**ndirect\_*Coombs* **A**ntiHumanglobulin **T**est (***IAT***)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Explain ***IAT*** MOA (2)
**IAT** = [**I**ndirect\_*Coombs **A**ntiHumanglobulin **T**est*] = ## Footnote ▶Uses [*IAT* and test blood] in lab to determine if h\_r serum contain [preformed IsoAgglutinins(IgM>IgG)] that agglutinate to common RBC antigens = “Antibody Screen” **▶I**ndirectly In Vitro: Mixes [test blood(which contain RBC loaded with common RBC antigens)] with host\_recipient's [plasma w/antibodies]. ⼀ The *IAT* will bind to any {h_r [Ab|compliment (IgM/IgG/C3d/C3/etc.)} that have inappropriately agglutinated to RBC common antigens→ *IAT* is visualized to identify the RBC antigens (if any) h_r Ab are sensitized to. * * * *[IAT = Indirect CAT]*: *CAT: *Coombs AntiHumanglobulin Test] // [g\_d: graft\_donor] // [h\_r: host\_recipient]*
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What is the purpose of the [**D**irect\_*Coombs* **A**ntiHumanglobulin **T**est (***DAT***)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Explain the ***DAT***MOA (3)
**DAT** = [**D**irect\_*Coombs **A**ntiHumanglobulin **T**est*] = ## Footnote *[CAT*: *Coombs AntiHumanglobulin Test] // [gd\ : graft\_donor] // [hr\: host\_recipient]* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ **DAT** ▶Uses *CAT to determine* if [gd\RBC] are *_ACTIVELY_* being Sensitized (and ultimately hemolyzed) by [Transfusion/Drug-Induced/ hr\Ig**_M_**] * * * ▶**D**IRECTLY EN VIVO: host\_recipient blood is taken and given *CAT* **D**IRECTLY → which will bind to (and allow us to visualize/identify) any {[hr\Ab|compliment (IgM>>IgG/C3d/C3/etc)] *_ACTIVELY_* agglutinating gd\RBC} ▶[⊕DAT is ALWAYS ABNORMAL] and usually comes from [host\_recipient Ig**_M_** *and* compliment] *(*but incomplete DAT comes from IgG | compliment acting alone)*
138
What conditions would you expect to find a *positive* [**D**irect\_*Coombs* **A**ntiHumanglobulin **T**est (***DAT***)] (5)
**DAT** = [**D**irect\_*Coombs **A**ntiHumanglobulin **T**est*] = ## Footnote *[CAT*: *Coombs AntiHumanglobulin Test] // [gd\ : graft\_donor] // [hr\: host\_recipient]* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [⊕DAT occurs in \_\_\_] - [Acute Intravascular Transfusion Hemolysis] - [Delayed IgG EXtravascular Transfusion Hemolysis] - [Drug-Induced IgG EXtravascular Transfusion Hemolysis] - [Autoimmune Hemolysis] - [HDN (Hemolytic Disease of Newborn)]
139
# During Blood Transfusion both Anaphylaxis and Acute Hemolytic Reactions can occur within minutes How do you differentiate the two based on: *Onset* *Cause*
*≤minutes*[Anaphylaxis (IgA-deficient pts' anti-IgA antibodies attack donor IgA)] = [Respiratory Distress, Angioedema]tx = Epi, antihistamine, CTS, [CardioPulm support] * * * *≤1 hr*[Acute Hemolytic Reaction (ABO mismatch 2/2 clerical error vs chronic transfusions) → [Preformed IgM using compliment (will have ⊕DAT)] → Intravascular hemolysis ] = [**T**achycardia/**L**umbarPain/**H**gbnuria/**B**leeding_hypOtension_DIC/**C**hestConstriction/**F**luLikeSx]tx = [NORMAL SALINE IVF]
140
# During Blood Transfusion both Anaphylaxis and Acute Hemolytic Reactions can occur within minutes How do you differentiate the two based on: * Sx* * Tx*
*≤minutes*[Anaphylaxis (IgA-deficient pts' anti-IgA antibodies attack donor IgA)] = [Respiratory Distress, Angioedema]tx = Epi, antihistamine, CTS, [CardioPulm support] * * * * ≤1 hr*[Acute Hemolytic Reaction (ABO mismatch 2/2 clerical error vs chronic transfusions) → [Preformed IgM using compliment (will have ⊕DAT)] → Intravascular hemolysis ] = [**T**achycardia/**L**umbarPain/**H**gbnuria/**B**leeding\_hypOtension\_DIC/**C**hestConstriction/**F**luLikeSx]tx = [NORMAL SALINE IVF]
141
[T or F] [Premedication with antihistamines and APAP] can prevent blood transfusion reactions
FALSE
142
[CLL-SLL] is a ⬜ type of CA , associated with 4 sx ( ⬜4), all to which indicate a worse prognosis * * * How is [CLL-SLL] diagnosis confirmed? (2) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *CLL-SLL: Chronic Lymphocytic Leukemia-Small Lymphocytic Lymphoma*
[monoclonal B-lymphocyte leukemia]; **LATO** sx **L**AD **A**nemia **T**hrombocytopenia **O**rganomegalyLiver/Spleen * * * [smudge cells(peripheral smear)] and [flow cytometry]
143
What should you remember regarding Biopsying Metastatic Cancer ? (4)
Patients with ▨[limited CA: solely Organ/Organ's lymph nodes] → limited bxorgan or organ LN] * * * ▩ [EXTENSIVE CA: metastasis/involves supraclavicular LN → EXTENDED bxof one of the metastasized sites instead [(supraclavicular (if involved)] > other sites] = this is because metastasized sites (like Supraclavicular LN excisional bx) tend to be ⬇︎invasive and ⬇︎complications for biopsy \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ ex: Pts with suspected metastatic Lung cancer (if possible)**should have initial bx from one of the metastatic sites(SCLV-if involved) > other**
144
Vancomycin Infusion Reaction
[**NON**-IgE] drug rxn involving vancomycin binding directly/activating mast cells → histamine release → [diffuse pruritus, erythema] ⼀proportional to infusion rate and resolved with d/c
145
# CO binds with greater affinity than O2 to Hgb and interferes with O2 offloading Describe the Symptom course for carbon Monoxide toxicity (9)
*sx: "Monixide hurts my [**MIND'S → SSSH**]* ​ **M**alaise ​ **I**rregularly SHARED HEADACHE (w/roommate, housemate, etc) ​ **N**ausea ​ **D**izziness ​ **S**kin ∆ [PINK-*like cYanide tox*] or [BLUE-*like methemoglobinemia tox*] * * * ​ **S**eizure/**S**yncope/**S**leepy-COMA/**H**eart❌ ## Footnote *tx: high-flow O2 via nonrebreather → hyperbaric oxygen*
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tx for Carbon Monoxide toxicity (2) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *sx: "Monoxide hurts my [MIND'S → SSSH*
[high flow O2 via nonrebreather] --*(if severe)*--\> hyperbaric oxygen
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Name the major causes of Carbon Monoxide toxicity (3) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What are 2 crucial diagnostics for Carbon Monoxide toxicity \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *sx: "Monoxide hurts my [MIND'S → SSSH]"*
148
# scombroid poisoning a. etiology * * * b. symptoms (6)
a. *(often confused for allergic rxn**) *in seafood stored at temperatures \> 15C, histidi**n**e undergoes decarboxylation → hista**M**ine = b. [flushing |throbbing HA|palpitations|abd cramps|diarrhea|(oral burning +/- “bitter taste”)] 10-30m after ingesting *(usually)_FISH_*
149
# Pufferfish Poisoning symptoms (3)
\*perioral tingling \*paralysis \*poor coordination
150
clinical features of Serum Sickness (3)
1. [type 3 immune complex-mediated hypersensitivity rxn] 2. primarily occurs after receiving [antibodies*Monoclonal/Chimeric*] or [antitoxins*heterologous*] 3. SSLR = gzd urticaria + feverlow grade + arthralgia + LAD \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *SSLR: Serum Sickness-Like Reaction*
151
Tx for [1º myelofibrosis (CIPM)] (2) | *CIPM: [Chronic Idiopathic Primary Myelofibrosis]*
{ [allogenic hematopoietic stem cell transplant] \< *[**age 60**]*\< [palliative care] }
152
Acute Leukemia will present with signs of \_\_\_\_\_ Which acute leukemia is associated with [Auer rods (eosinophilic inclusions)]?
**PAN**cytopenia ; [aPL ⼀acute PROMyelocytic Leukemia M3 (T15/17)] *Dx = smear showing [myeloblast\>20%] --\> flow cytometry for confirmation*
153
What are the toxicities for MTX?
154
St.John's wort is an OTC herbal supplement used alternatively for ⬜. Why should it be used with caution?
[mild/moderate depression] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ It **upregulates** [CYP P450] ➜ ⇪ metabolism ## Footnote **Chronic alcohol**ics **St**eal **Phen-Phen** & **N**ever **R**efuse **Gr**easy **Carb**s *which keeps them UP*
155
Octreotide is a ⬜ used to treat ⬜ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ explain how
[somatostatin 14 analogue] ; [Somatotrope - Functional Pituitary Adenoma] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ inhibits [pituitary somatotrope] from releasing Growth Hormone in a [functional pituitary adenoma]
156
What is [Diamond Blackfan anemia]? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ cp? -4
congenital bone marrow failure in infancy \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 1. absent thumbs 2. craniofacial abnormalities 3. [SEVERE Macrocytic anemia (hgb \< 9 at birth)] 4. reticulocytopenia
157
By 21 years old, a **fully immunized patient** should have ⬜ total [TETANUS toxoid vaccines] . Name the vaccines and what age they're given
6 *= (this is the expected # lifetime Tetanus vaccines)* *#5 if 18 yo* ## Footnote \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [DTaP = (2 / 4 / 6 / 15 ) months old] + [TDaP at 11 years old] *+ TDaP booster @ 21 years old*
158
What type of _psychiatric_ side effects does CTS have? - 4 | (CorTicoSteroids)
**S**teroids **M**ake **P**eople **D**epressed! 1. **S**uicidality 2. **M**ania 3. **P**sychosis 4. **D**epression
159
normal ALP level \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *ALP = Alkaline Phosphatase*
25 - 100
160
List the *Sexual* Side Effects of SSRI -3 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ How do you manage this? -2
"*SSRI... {Sexually Slows & Really Impairs LEO*" ⬇︎Libido | ⬇︎Orgasm | [⬇︎Ejaculation (delays)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ - [**SWITCH** to non-SSRI (buproprion/mirtazapine) ⚠️{DOSAGE reduce cautiously for pts on high-dose/long term SSRI)}] *or* - [**AUGMENT** with sildenafil]
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How do you manage newly diagnosed [LCIS (Lobular carcinoma in situ)] -2
LCIS is nonmalignant, but still has ⇪ risk for development into [invasive breast CA or DCIS] = **excisional biopsy** + lifetime surveillance
162
*pt s/p PE just started Heparin but develops HIT* What's the first step for suspected [Heparin Induced Thrombocytopenia (HIT)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ when is management with Warfarin typically ok to start after HIT ?
d/c **ALL**forms of Heparin ➜ alternate anticoagulants (i.e. *direct thrombin inhibitors*) [even if no thrombosis present] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ platelet \> 150K
163
Disulfiram MOA \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ How do you decide if you should give a patient Disulfiram or Naltrexone?
## Footnote [inhibits aldehyde dehydrogenase ➜ [**SEVERE NV** with any _EtOH intake_] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *Disulfiram for* [Die-Hard ABSTINENTS who want to stay Abstinent] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Naltrexone ( ⬇︎EtOH cravings) for [moderate/SEVERE Alcoholism in opioid-free patients that are Alcoholic] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *[acamprosate (glutamate modulator)] is also used in Alcoholism*
164
⬜ is the GREATEST risk factor for Male Breast Cancer. ⬜ is the second greatest risk factor for Male Breast Cancer. And ⬜ is the third greatest risk factor for Male Breast Cancer \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Etx for Greatest risk factor? ; etx for 2nd greatest risk factor?
BRCA mutation \> \> \> Klinefelter Syndrome ## Footnote \> [**LAME** (**L**iver failure/(**A**lways Eating {Obese})/**M**arijuana/(**E**strogen:androgen ratio ⇪ {gynecomastia})] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ BRCA = auto DOM mutations ➜ [⇪ Male Breast CA risk x 100] [klinefelter syndrome XXY] = male having extra "X" chromo ➜ [Estrogen:androgen ratio ⇪] ➜ [⇪ Male Breast CA risk x 20]
165
normal blood glucose is ⬜ What is Whipple's triad and what does it indicate?
60-100 ## Footnote *(some can go down to 45 with no sx)* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Whipples = [low BG] + [*PUSH* low BG sx] + [*PUSH* low BG sx improve after glucose administration] = **true hypOglycemia** \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *hypOglycemia sx = need **PISH** juice = **P**alpitations/**U**neasy Irritable/**S**weating/**H**A*
166
how do you prevent Tumor Lysis Syndrome? - 2 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ how do you *treat* Tumor Lysis Syndrome (with AKI) -2? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Why do these differ?
**AF** ➜ **RF** px = **A****F**: [**A**llopurinol (xanthine oxidase inhibitor)] +**F**luids IV] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ TX (for AKI 2/2 TLS) = **RF**: [**R**asburicase (urate oxidase analogue)] + **F**luids IV] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ **[A**llopurinol prevents *A*dditional serum uric acid formation] whilst [**R**asburicase metabolizes *R*ealtime (already existing) serum uric acid]
167
the presence of [HbA 60% : HbS 40%] on electrophoresis is c/w ⬜ . What are the subsequent sx of this?
Sickle Cell **TRAIT** = ASYMPTOMATIC (does not cause Anemia)
168
Anemia of Chronic Disease MOD (5)
⭐caused by **IL6 chronic inflammation** → liver secretes hepcidin → [blocks 🆚 suspend] {[DJ enterocyte *ferroportin*] and [macrophage *ferroportin*]} from *transfering* iron from FerriTin*storage* to Transferrin*transport**(→🔻TS(if ferroportin _blocked_) 🆚 normal TS(if ferroportin only _suspended_))*, ⭐and exact opposite for FerriTin= 🔺*(ferroportin _blocked_) * 🆚 nml*(ferroportin suspended* FerriTin ⭐but additionally❗️ all this hoopla also *paradoxically* keeps Transferrin out of circulation (*which → ⬇︎TIBC (but with 🔻/normalTransferrin Saturation)) = rare*\* ⭐AND ULTIMATELY this ➜ {[(Normo*initially*→micro*later*)cytic] normochromic anemia} | \*Typically *Transferrin Saturation* and *TIBC* are inverse related ## Footnote so.... \_\_\_\_\_\_\_\_\_=\_\_\_\_\_\_\_\_ *iron studies =*{[**↧**Transferrin Saturation **with** *paradoxic* ⬇︎TIBC]\*, [**↥**FerriTin], [⬇︎iron*in circulation**iron in circulation is low because it's still being sequestered to FerriTin by Hepcidin *]} = {[**🢗**MCV*Normo --(to)--> micro* -CYTIC ] [**Normo**CHROMIC] } \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_x\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *🔎DJ = Duodenal-Jejunal* *🔎↧ = (DEC or normal)* *🔎↥ = (INC or normal)* *🔎TS = Transferrin Saturation*
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at what hgb should you consider blood transfusion?
hgb \< 7
170
Why are abx **NOT** used in treating Hemolytic Uremic Syndrome? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the management for Hemolytic Uremic Syndrome? *HUS HAT*
killing bacteria could ➜ ⇪ release of Shiga toxin \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ SUPPORTIVE CARE ONLY (*fluid/electrolyte mgmt | blood transfusions | dialysis*)
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Tetrabenazine ## Footnote *MOA* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *Indication*
[ dopamine🟥 ] ; Huntington's disease
172
*Stimulant toxicity and Anticholinergic toxicity have a lot of sx overlap* What symptom helps to differentiate the two?
SKIN \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ **S**weating = **S**timulant tox **A**LL Dry = **A**nticholinergic tox
173
[Cryoglobulinemia Type 1] MOD -2
⭐[B Cell CA (Multiple Myeloma)] ➜ Monoclonal immunoglobulins that aggregate at low temp \< 37C = Cryoglobulins. ⭐Cryoglobulin precipitation ➜ noninflammatory microvascular occlusion and hyperviscosity sx when CG are high ## Footnote *hyperVIScosity sx = Vertigo, Imbalance/ataxia, Sight blurry*
174
[T or F] Varenicline has many serious adverse effects when combined with Nicotine Replacement Therapy
FALSE \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *Varenicline + NRT = ✔︎*
175
features of Multiple Myeloma -4
osteoclast activating factor ➜**CUBP***sx* 1.[(***C**RAB*) - end organ damage] *hyper**c**alcemia*(→ vertebral dark lytic lesions/bone fx, constipation, depression) *R*enal failure(2/2 Ig and Bence Jones proteinuria --\> DEATH) *A*nemia normocytic [*B*ack pain i\ vertebral dark lytic lesions and bone fx] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 2.[**U**rine IgG or Urine IgA] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 3.[**B**one marrow with ≥10% clonal plasmacytosis/plasmacytoma (*If Infection → DEATH!*)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 4.[**P**rotein (M Protein) in serum]
176
[CRYOGLOBULINEMIA TYPE 2] MOD
[Chronic Viremia/Autoimmune disease] ➜ [B cell hyperactivation] ➜ forms IgM which bind to IgG = ⇪ mixed circulating immune complexes these [circulating immune complexes] desposit in small vessels ➜ INFLAMMATORY vasculitis (*glomerulonephritis*) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
177
What 3 physical exam findings indicate **widespread microvascular occlusion**?
1. Livedo reticularis 2. digital ischemia 3. retiform purpura (net like reflect of vasculature - *image*)
178
[Febrile nonhemolytic transfusion rxn] occurs ⬜ after transfusion starts. and Pts have what sx -3? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ How is this related to Leukoreduction? -3
**[1H-6H]** ; [Fever / Chills / Malaise] (NO HEMOLYSIS) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Leukoreduction = ⬇︎[Febrile nonhemolytic transfusion rxn] ⬇︎HLA alloimmunization✳ [⬇︎CMV transmission *(since CMV resides in Leukocytes)*] ## Footnote ✳alloimmunization: = *host_recipient immune response to otherwise [foreign antigens (blood group ag|histocompatibility ag) from ANOTHER PERSON] [⼀usually from incidental exposure like transfusion] *
179
[Glucose-6-Phosphate Dehydrogenase] deficiency cp -3
[HEMOLYSIS WITH UNCONJUGATED HYPERBILIRUBINEMIA → JAUNDICE] [SUDDEN BACK PAIN] FATIGUE \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *X-linked RBC G6PD defect in African/Middle Eastern/Southeast asian ➜ ⇪ RBC hemolysis from* [**DASANI** H2O2 oxidant stress]
180
[Glucose-6-Phosphate Dehydrogenase] deficiency etx -5
✏️ [X-linked RBC (G6PD\_Glutathione reductase) deficiency] in African/Middle E/SE asian * * * ✏️= ["**DASANI**" H2O2 oxidant stress] → [RBC H2O2*(since there's no G6PD to drive GSH buffer rxn which normally takes up oxidant stress)*] → [Oxidation denaturation of Hgb Sulfhydryl groups] = Formation of Heinz bodies in RBC→ [RBC damage and ⬇︎ membrane flexibility] = sx : * * * ⬇︎ ✏️a.. 🅸1 = Intravascular hemolysis from [*Oxidized denatured Hgb Sulfhydryl group* membrane damage] → *[HEMOLYSIS WITH **UN**CONJUGATED HYPERBILIRUBINEMIA → JAUNDICE]** ✏️b.🅸2: [Heinz body RBC*(RBC with oxidized denatured Hgb Sulfhydryl groups)*] travel thru splenic cord and splenic macrophages BITE out Heinz body] → [Bite *RBC*] --(eventually after enough bites) forms into→unstable Spherocyte} → 🅸 ✏️c. 🄴Xtravascular hemolysis from [Heinz body-RBC] suddenly getting stuck in spleen → removed by splenic macrophages → [*SUDDEN BACK PAIN] + FATIGUE* ## Footnote *🔎 🅸 = Intravascular hemolysis from membrane damage → [HEMOLYSIS WITH **UN**CONJUGATED HYPERBILIRUBINEMIA → JAUNDICE]**
181
[Glucose-6-Phosphate Dehydrogenase] deficiency How do you diagnose this? -3
[X-linked RBC (G6PD\_Glutathione reductase) defect] in African/Middle E/SE asian * * * 1.[measure G6PD activity] 2.[peripheral smear:bite RBCs] 3.[peripheral smear:[heinz body RBCs] (= accumulated “**SHODE** =byproducts of INC H2O2 on RBC) ] *** **SHODE**: [Sulfhydryl Hgb ⼀ *Oxidized, Denatured* entities]
182
diagnosis?
[Heinz body RBCs*(accumulated oxidized denatured hgb sulfhydryl groups)*] in **G6PD** ## Footnote ***GLUCOSE 6 PHOSPHATE DEFICIENCY***
183
Diagnosis? | What's the best diagnostic test for this condition?
**Spherocytes**(Hereditary Spherocytosis | G6PD) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Osmotic Fragility test
184
labs for Thalassemia *trait* -5 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ How do you differentiate *α* from *β* Thalassemia?
1. anemia that is 2. ⼀microcytic (*low mean corpuscular volume*) 3. ⼀hypOchromic (*low mean corpuscular hgb*) 4. [⇪ ferriTin (*INC RBC turnover ➜ more iron to Tuck into storage*)] 5. [normal RDW (*all RBC uniformly made microcytic*)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Hgb electrophoresis: [βT minor = ⇪ Hgb**A2**]
185
[Gp2b/3A R blockers] are anti-⬜ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Name all 3
anti-**platelet** **\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_** ***"**I **ATE** [Gp2b/3A] for breakfast"* [**A**BCiximab / **T**irofiban / **E**pTiFibatide]
186
Describe Management for Tetanus Prophylaxis
187
# acanth Polycythemia is hgb of ⬜ in Men and ⬜ in Women. What's the first step to evaluating polycythemia? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ How do you interpret this data? -2
hgb: M \>18.5 | W\>16.5 measure Erythropoietin \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *polycythemia? ➜ [**low EPO = polycythemia vera] vs [HIGH EPO = 2° polycythemia (chronic hypoxia or Renal Cell Carcinoma)]*
188
Polycythemia is ⬜ in Men and ⬜ in Women. measuring EPO is first step in working up Polycythemia \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ ⬜ is the most common cause of 2° polycythemia.
hgb: M \>18.5 | W\>16.5 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ CHRONIC HYPOXIA (consider carboxyhgb and sleep apnea) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *polycythemia? ➜ [**low EPO = polycythemia vera] vs [HIGH EPO = 2° polycythemia (chronic hypoxia or Renal Cell Carcinoma)]*
189
s/s Zinc Deficiency -4 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *must be confirmed by lab*
Doesn't grow Diaper rash Dermatitis perioral Diarrhea
190
Organophosphate poisoning MOD \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Tx? (2)
ACh**E** inhibitor --\> TOO MUCH ACh in cleft --\> [“*OTC* **dUMBBELLS C**reate **M**uscle”] ? …….**PAID** * Organophospahtes are used in Agricultural Pesticides* * TX = Atropine + [Pralidoxime (reactivates ACh**E**)]*
191
What's the 1ST medication given for Atheroslcerosis? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ When is it actually indicated to give? (3)
STATIN \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ * [LDL ≥190] * [age ≥40 + DM] * [(10y ASCVD risk) ≥7.5%] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *THIS IS REGARDLESS OF BASELINE LDL*
192
Chronic Granulomatous Disease MOD (3)
▶[X-linked **recessive** 1° immunodeficiency] ▶defect in [neutrophil (NADPH oxidase*resp. burst*)] ➜ impairs neutrophil superoxide formation ➜ [impaired neutrophil intracellular killing(neutrophils can eat BUT NOT KILL)] ▶ = defective granuloma formation ➜ recurrent [catalase positive infections*from "**SPACE B**ugs"*] (**Aspergillus** = MAJOR COD / **Staph A**=liver/skin abscess/adenitis) ## Footnote ▶*(catalase+) infections = **SPACE B**ugsStaphA&Serratia/Pseudomonas/Aspergillus&Nocardia/Candida/Enterobacter/Burkholderia-cepacia
193
[Chronic Granuloma Disease] management? (3)
[(SMXTMP) + itraconazole]px \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [interferon-2]tx ## Footnote ▶*(catalase+) infections =* **SPACE B**ugs*StaphA&Serratia/Pseudomonas/Aspergillus&Nocardia/Candida/Enterobacter/Burkholderia-cepacia* ▶*dx = nitroblue tetrazolium test*
194
Name the most common [Catalase POSITIVE] organisms (5) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Which 2 are related to Chronic Granulomatous Disease? and how?
▶*(catalase+) infections = **SPACE B**ugs_StaphA_&Serratia/Pseudomonas/_Aspergillus_&Nocardia/Candida/Enterobacter/Burkholderia-cepacia*dx = nitroblue tetrazolium test*
195
Describe the process of EtOH breakdown to Acetic Acid and explain how Metronidazole disrupts this
Metronidazole has Disulfiram-like activity --\> *(toxic)*Acetaldehyde accumulation --\> Flushing/NV/Cramps after drinking
196
Describe pathophysiology for acute APAP OD \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Describe the 4 clinical stages of APAP OD
197
How do you diagnose APAP OD? (2) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ ⬜ and [N-AcetylCysteine] are the 2 mainstays of treatment. When is NAC most effective? *DELAYED HOSPITAL PRESENTATION = WORST OUTCOME*
[Activated Charcoal (if within 4 h post ingestion)] ## Footnote *NAC is most effective when given **within 8 hours post ingestion** prior to hepatoxicity*
198
MOD for APAP overdose \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ ⬜ and [N-acetylcysteine] are mainstay treatments, but NAC is most effective if given ⬜. How does NAC work? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *DELAYED HOSPITAL PRESENTATION = WORST OUTCOME*
during APAP OD, APAP --(*via CYP**2E1***)--\> [toxic NAPQI] and [toxic NAPQI] depletes intrahepatic glutathione as it's ➜ [NON-TOXIC cysteine & mercapturic acid] once intrahepatic glutathione is all depleted [toxic NAPQI] accumulates ➜ hepatotoxicity \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [Activated Charcoal (if within 4h post ingestion)]- *binds APAP* [NAC (most effective within 8 hrs post ingestion)] ⇪ intrahepatic glutathione and restores [(toxic NAPQI) ➜ (nontoxic cysteine & mercapturic acid)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *protective \< [EtOH chronicity] \< exacerbant*
199
MOD for APAP overdose \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ How does EtOH affect this process? (2) *DELAYED HOSPITAL PRESENTATION = WORST OUTCOME*
during APAP OD, APAP --(*via CYP**2E1***)--\> [toxic NAPQI] and [toxic NAPQI] depletes intrahepatic glutathione as it's ➜ [NON-TOXIC cysteine & mercapturic acid] once intrahepatic glutathione is all depleted [toxic NAPQI] accumulates ➜ hepatotoxicity \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *acute EtOH competes for *CYP**2E1*** ➜ DEC [toxic NAPQI] = protective* *CHRON**IC** EtOH upregulates *CYP**2E1*** ➜ **INC** [toxic NAPQI] = exacerbant* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [Activated Charcoal (if within 4h post ingestion)]- *binds APAP* [NAC (most effective within 8 hrs post APAP ingestion)] ⇪ intrahepatic glutathione and restores [(toxic NAPQI) ➜ (nontoxic cysteine & mercapturic acid)]
200
How does Alcoholic ketoacidosis clinically present? (4) how is this different from DKA? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ tx for Alcoholic ketoacidosis? -2
*suspected Alcoholic with:* AG met acidosis INC osmolar gap ⊕ketones variable blood glucose (DKA has BG \> 250) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ tx = [THIAMINE**1ST** → Dextrose IVF2nd] | *dextrose will➜ insulin secretion ➜ metabolism of ketone bodies to HCO3*
201
*iron deficiency anemia and thalassemia both cause microcytic anemia* What's a labatory method for differentiating them?
*MentzeR Index = MCV/RBC* thalassemia \< [MentzeR Index of 13] \< IRON DEFICIENCY ANEMIA \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_
202
Why does Carcinoid Syndrome cause Niacin B3 deficiency?
Carcinoid tumors utilize Tryptophan to secrete tons of Serotonin. Tryptophan is also needed to make Niacin B3. This can --\> Pellagra DDDD ## Footnote *Do not confuse Carcinoid Syndrome with VIPoma which presents similarly but VI**P**oma affects **P**ancreas while Carcinoid affects small intestine*
203
*Clozapine's SE is agranulocytosis* Name the Granulocytes - 3
**BEN** **B**asophils **E**osinophils **N**eutrophils *Clozapine also causes Metabolic Syndrome X, Seizures and Myocarditis*
204
Imiquimod indications (3)
1. [Actinic⼀Solar Keratosis] 2. [HAWCA] 3. [superficialBCC] ## Footnote *🔎HAWCA = HPV Anogenital Warts Condylomata Acuminata* || *🔎BCC = Basal Cell Carcinoma*
205
Buspirone MOA \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ indication
Buspir**one** = [5HT**1**α R partial agonist] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ GAD \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *[slow onset] and [lacks muscle relaxant/anticonvulsant properties]*
206
cp for carbon Monoxide poisoning?-9 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Dx?
*sx: "Monixide hurts my [**MIND'S → SSSH**]* **M**alaise **I**rregularly SHARED HEADACHE (w/roommate, housemate, etc) **N**ausea **D**izziness **S**kin ∆ [PINK-*like cYanide tox*] or [BLUE-*like methemoglobinemia tox*] * * * **S**eizure/**S**yncope/**S**leepy-COMA/**H**eart❌ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Dx = [ABG Carboxyhemoglobin levels] ## Footnote ⚠️ Monoxide tox can present very similarly to [cYanide tox (which is also [PINK SKIN **BUT with Yucky bitter almond breath**)] and [Methemoglobinemia tox (BLUE SKIN)] so be careful!
207
toxicity of what 2 substances causes PINK SKIN?
{carbon Monoxide tox*(MIND'S → SSSH)* ➜ [PINK SKIN]} \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ {C**Y**anide tox ➜ [PINK SKIN with **Y**ucky **BITTER ALMOND BREATH**]}
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Houses built before what year ⬆︎ risk for lead poisoning?
1978 ## Footnote *false positives occur so be sure to confirm with venous blood draw!*
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Antidote for CYanide toxicity - 4
"cYaMonoxide toxicity **N**eeds **O**vert **S**moke **H**elp" *empiric tx for cYanide and (carbon)Monoxide toxicity* 1. [**N**itrites*(induces methemoglobinemia)*] 2. **O**xygen 100% (*CO tx*) 3. [**S**odium thiosulfate] 4. [**H**ydroxoCoBalamin(*binds cYanide ➜ excretable cYanocobalamin*)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *empircally treat smoke inhalation pts for **CY**anide tox!*
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[SEVERE \<28C (or unresponsive Moderate) HYPOTHERMIA] management \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ sx? (3)
[SEVERE \<28C (or unresponsive Moderate)]: [ACTIVE CORE REWARMING *(WARM PERITONEAL LAVAGE, WARM HUMIDIFIED O2)*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ {*"(_C_ut ) BLM!"*--tx--> [ACTIVE CORE🔥]} | BLM = Brain/Liver&heart/Muscle ## Footnote {*"(Cut) BLM"*--tx--> [ACTIVE CORE🔥]} = 🧠 *Brain*= _C_OMA 🫁*Lungs/heart* = _C_ardiac _Vt_ arrhythmia 💪*Muscle* = _C_V Collapse
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[Moderate 28-32C (or unresponsive mild) hypOthermia] management \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ sx? (5)
[Moderate 28-32C (or unresponsive mild) hypOthermia]: [ACTIVE e🅇ternal warming *(heated blankets, heated baths, heated forced air) *] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ {*"(slow) BLM"*--tx--> [ACTIVE🅇🔥]} | BLM = Brain, [Lungs&heart, Muscle] || 🅇=E🅇ternal ## Footnote {*"(slow) BLM"*--tx--> [ACTIVE🅇🔥]} = 🧠 *Brain*lethargy [🫁&🩷]*Lungs&heart*hypOventilation, bradycardia/_atrial_ arrhythmia, 💪*Muscle*DEC shivering
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[mild 32-35C hypOthermia] management \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ sx? (5)
[mild 32-35C hypOthermia]: [passive external warming *( warm blankets & remove wet clothes)*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ {*"(FAST) BLM"*--tx--> [passive🅇🔥]} | BLM = Brain, [Lungs&heart, Muscle] || 🅇=E🅇ternal ## Footnote {*"(FAST) BLM"*--tx--> [passive🅇🔥]} = 🧠 *Brain*ataxia, dysarthria 🫁*Lungs/heart*tachypnea/tachycardia 💪*Muscle*INC shivering
213
a. tPA MOA (3) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ b. What other 2 agents have same MOA? c. Which agent has *opposite* MOA?
b. Streptokinase , Urokinase c. Aminocaproic acid
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Kava Kava is a supplement used for (⬜2) What is its major side effect?
[*(_mild_ ⬇︎ only)*anxiety][*(_mild_ ⬇︎ only)*insomnia ] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [LIVER FAILURE weeks after starting it]
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What is the treatment for ASA toxicity? (2) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ MOA for this tx?
[**NaHCO3 IV**] ➜ [HemoDialysis (if severe)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [NaHCO3 IV] Alkalinizes Blood and Urine ➜ basic blood/urine environment ➜ deprotonation of Salicylates ➜[Salicylate *ion*] inability to be reabsorbed into CNS or into [blood from renal tubules] ➜ [⇪ salicylate ion excretion] *severe = cerebral/pulmonary edema | renal failure*
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elevated lead level on capillary testing must be confirmed by ⬜
venous lead level \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *lead tox ➜* *neurobehavioral impairment. tx = AVOID LEAD EXPOSURE*
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what are the hallmarks of Splenic Vein Thrombosis? - 2
1. **[isolated** stomach fundal varices] -\> variceal hematemesis 2. [splenomegaly] -\> anemia/thrombocytopenia ## Footnote *Splenic Vein Thrombosis is commonly a/w pancreatitis*
218
Why is Succinylcholine contraindicated in pts with burns, myopathies, crush injuries or denervating Dz
Can cause **SIGNIFICANT K+ RELEASE --\> VFIB** in pts at high risk for Hyperkalemia
219
How many ATP are yielded in Aerobic vs. AnAerobic metabolism?
Aerobic = **32** AnAerobic = [2 + Lactate]
220
Multiple Myeloma MOD (5)
[FGFR3/cyclin D1 and D3 gene defects] → abnml [*bone marrow* monoclonal plasma cell proliferation] → produces useless immunoglobulin (IgG , IgA) --\> [Osteoclast Activating Factor] which → **CUBP** | *When MM produces IgM = WaldenstroM Macroglobulinemia* ## Footnote **CUBP** 1.{[**C***RAB* end organ ❌](hyper*C*alcemia), *R*enal Failure 2/2 bence jones proteinuria, *A*nemia_normocytic, *B*ack pain i\lytic bone fx } \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 2.[**U**rine IgG/IgA]*get urine immunoelectrophoresis* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 3.⚠️ [**B**one marrow >10% plasmacytosis]CONFIRMS MM DX \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 4.[**P**rotein(*serum:* M protein, IgG, IgA [monoclonal "M" spike if MGUS & *smear:* Rouleaux )]
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Multiple Myeloma dx - 4
**CUBP** 1.{[**C***RAB* end organ ❌](hyper*C*alcemia), *R*enal Failure 2/2 bence jones proteinuria, *A*nemia_normocytic, *B*ack pain i\lytic bone fx } \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 2.[**U**rine IgG/IgA]*get urine immunoelectrophoresis* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 3.⚠️ [**B**one marrow >10% plasmacytosis]CONFIRMS MM DX \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 4.[**P**rotein(*serum:* M protein, IgG, IgA [monoclonal "M" spike if MGUS & *smear:* Rouleaux )]
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*Spinal Cord Compression can be from DJD, Epidural Abscess or Tumor* Which Cancer metastasis are associated with Tumor Spinal Cord Compression? - 5
1. Prostate 2. Renal 3. Lung 4. Breast 5. Multiple Myeloma
223
Wernicke-Korsakoff syndrome tx
[Thiamine B1]
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Why does [Thiamine B1] deficiency cause low ______ ?
**ATP**; *Think **ATP***: [Thiamine B1] is needed to catabolize glucose → **ATP** with the "*αTP*" enzymes : [α-ketoglutarate dehydrogenase (TCA)], [Transketolase (HMP shunt)], [Pyruvate dehydrogenase (TCA)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 💡so... if [Thiamine B1] deficient ➜ "*αTP*" enzyme [impaired glucose metabolism → ATP depletion] which → [Wernicke Korsakoff Syndrome] and [BeriBeri] ## Footnote 📖[**BeriBeri** falls into 3 subtypes (*WET, DRY, BOTH)*] : 1.[High Output Dilated HF + edema] = ***WET*** 2.[Symmetrical Peripheral Neuropathy + muscle wasting] = ***DRY*** 3.[***WET*** and ***DRY***]*(BOTH)*
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a. acute *TOXIC* ingestion of ASA = taking [__________mg/kg in 1 dose]. \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ B. *TOXIC* ASA ingestion will lead to what 3 acid-base disturbances?
a. \>100 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ b. 1st: ASA-induced[AG metabolic acidosis] ➜ 2nd:*compensatory*[respiratory alkalosis] = 3rd: [MIXED *AG metabolic acidosis* WITH respiratory alkalosis] | "***A**ir HOT/[**S**ound ringing-stomach sick]/**A**ir fast*"
226
quick way to distinguish acute Aspirin OD from acute Acetaminophen OD?
_Acute_ Aspirin ingestion ➜ **vomiting** | "*acute A _S_ A* gets you *_S_*ick to ur Stomach(vomiting)!"
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classic triad for Aspirin OD
**ASA** --\> [Mixed Respiratory alkalosis + AG metabolic acidosis] (Normal pH/DEC PCO2/DEC HCO3) 1. [**A**ir is Fast (*Tachypnea*)] 2. {[**S**ound ringing (*Tinnitus*)] ... and [Stomach Sick (*Vomiting*)]} 3. [**A**ir is Hot (*Fever*]
228
Gold standard for TIA prophylaxis ? How does it work?
ASA; COX inhibitor --\> DEC [Thromboxane A2] --\> DEC Platelet **aggregation** and **vasoconstriction** ## Footnote ✏️*Activated platelet → secretes fibrinogen and {[ThromboxaneA2 & ADP] → [INC (Gp2b/3a\_platelet fibrinogen R) expression on platelets]}*
229
Explain Winter's Formula -4
⏸️[COMPENSATED arterial PaCO2*(compensating for acute metabolic∆|acute renal∆)*] ⏸️*should* be within ⏯️ {***+/- 2 of*****[1.5 x HCO3 + 8]**} ... ⏏️...If not = [*mixed* acid/base picture]
230
Tx for [*unknown* Overdose Ingestion] -5
*"treating unknown OD ingestion is a **BITCH**"* 1. **B**icarb IV (NaHCO3 IV → ⇪ ASA excretion)✏️ 2. [**i**Pecac syrup (if alert)]= induces emesis 3. **T**oss out Gastric contents with [Gastric lavage (if within 1 hr of ingestion)] 4. [**C**harcoal activated] = absorbs ingested toxin 5. **H**emodialysis ## Footnote 📝<(NaHCO3 IV alkalinizes urine and blood → deprotonates salicylate → salicylate *ion* can't cross membrane = no renal reabsorption or BBB crossing
231
What lab abnormalities indicate Hemodialysis to treat Aspirin OD -3
1. [initial salicylate \> 160] mg/dL 2. [6 hr salicylate \> 130] 3. persistent acidosis pH \< 7.1
232
major symptoms of TCA OD (amitriptyline) -6
Anticholinergism! *1.mad\hatter{delirium, seizure}, 2.blind\bat, 3.dry\bone{dry mouth} 4.hot\hare{HYPERthermia} 5.bowel-bladder lose [contraction] tone{urinary retention} 6. heart runs **LONG***{[6a. Widened QRS \> 100ms ➜ Arrhythmia]*NaHCO3IVtx*] & 6b.Prolonged intervals}
233
TCA OD treatment (4)
* [NaHCO3]IV = for QRS widening or Ventricular arrhythmia * Activated charcoal **if within 2h ingestion** * Intubation/oxygenation * IVF
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MOA for Sodium Bicarb in TCA OD
In Cardiac tissue, *Sodium Bicarb* [⬆︎extracellular sodium] and [⬆︎ extracellular pH] to alleviate TCA's cardio-depressant action on sodium channels
235
In pts with TCA overdose, what's the most important vital to monitor ? why?
**QRS duration** \_\_\_\_\_\_\_\_\_\_\_ QRS \> 100ms --\> INC Vt arrhythmias and seizures (tx: NaHO3IV)
236
how long is [1st episode DVT/PE] tx with [Factor 10A inhibitors]?
≥3mo
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# DVT/PE are treated with [*rafX* Factor 10a inhibitor] or [Warfarin] Compare the following parameters between [*rafX* Factor 10a inhibitor] and [Warfarin] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ a. Mechanism of Action b. Therapeutic onset c. Overlap needed? D. Laboratory monitoring
## Footnote DVT #1 = {[*rafX* Factor 10a inhibitor] x ≥3mo}tx
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T or F \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ "[Antiplatelets (like ASA, CLOpidogrel, ABCiximab)] are NOT used in DVT tx"
TRUE
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T or F "Patients who develop [HITT2 or HITT1] can use Heparin after 6 months"
**FALSE!!!!!!!!** PATIENTS WITH HITT ARE HEPARIN-BANNED FOR LIFE!!
240
Name the [Direct Thrombin Factor 2a inhibitors] (3)
**DAB** 1. **D**abigatran 2. **A**rgatroban 3. **B**ivalirudin ## Footnote -bivalirudin is related to hirudin (anticoagulant leeches use) -Use [Direct Thrombin Factor 2a inhibitors] in patients with HIT
241
mgmt for HITT2 -3 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *HITT2 = Heparin Induced Thrombocytopenia type 2*
1. DISCONTINUE ANY HEPARIN 2. start *DAB*[Direct Thrombin Factor 2a inhibitors] or 3. start [fondaparinuX(factor 10a inhibitor)]
242
How do you calculate liklihood of [HITT2 (Heparin Induced Thrombocytopenia type 2)]? -4
HITT2 = all 4T score 1. **T**hrombocyte DEC GOE30-50% 2. **T**iming onset 5-10d after Heparin exposure (or 1d if previous exposure) 3. **T**hrombosis present 4. **T**ook Out other thrombocytopenia causes
243
[Heparin Induced Thrombocytopenia type1 (HITT1)] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ mechanism of disease
[HITT1] = **NONIMMUNE** Heparin-Platelet clumping] ➜ [platelet \> 100K] and resolves spontaneously
244
"oozing from mucosal sites" is associated with which: HITT or DIC?
DIC
245
[Heparin Induced Thrombocytopenia Type2 (HITT2)] \_\_\_\_\_\_\_\_ mechanism of disease -3
246
Leukocoria = _____ associated with what kind of Cancer?
WHITE EYE REFLEX; Retinoblastoma
247
Is Haptoglobin ⬆︎ or ⬇︎ in Hemolytic Anemia? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Why?
**DECREASED** **\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_** Liberated Hgb (after RBC hemolysis) BINDS to serum Haptoglobin --\> HgbHaptoglobin complex --\> Cleared by Liver *Haptoglobin picks up Haphazard hgb*
248
T or F: Brain Metastasis from NonSOLC is Chemo**sensitive**
FALSE! *NonSOLC brain metz = NONchemoSensitive*
249
CP of Acute Intermittent Porphyria - 3
A-I-P causes **N-A-P** 1. Psychosis *acute onset* 2. **Abd pain *acute onset*** 3. Neuropathy *acute onset* ​*Fam hx of this is VERY suggestive of AIP*
250
In Order, List the 5 Enzymes involved in Heme Synthesis
*AAPUF* 1st: \<**A**LAS | Sideroblastic\> 2nd: \<**A**LAD | lead tox\> 3rd: \<**P**orphoDeam | AIP\> 4th: \<**U**ROPorphoDeCARB | PCT\> 5th: \<**F**errocheletase | lead tox\> \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *enzyme❌is associated with [| _⬜_]*
251
isolated ELEVATED IRON is specific to what type of anemia?
Sideroblastic anemia
252
Anemia with normal iron studies is specific for what type of anemia?
Thalassemia (except 3 gene deletion alpha thalassemia) ## Footnote *Dx = Hgb electrophoresis with genetic studies if alpha thalassemia*
253
Tx for sideroblastic anemia
Pyridoxine B6
254
Causes of Vitamin B12 deficiency - 6
1. **PERNICIOUS ANEMIA = MOST COMMON CAUSE** 2. Vegan/Vegetarian 3. Blind loop syndrome (Gastrectomy or RYGB) 4. Diphyllobothrium latum 5. Pancreatic Insufficiency 6. Terminal iLeum damage (Crohns)
255
how do you differentiate Vitamin B12 deficiency from Folate deficiency
Folate deficiency has [NO CNS🧠∆] -------- Vitamin B12 isomerizes **methymalonyl coA** in the spinal cord myelin ---\> succinyl coA. Without it --\> suBACute combined degeneration *usually manifest as paresthesia/peripheral neuropathy*
256
Chronic hemolysis is associated with what type of gallstones?
**Pigmented bilirubin** gallstones
257
# Peripheral Smear of pt with Sickle Cell Disease Explain this abnormal finding
Howell Jolly Bodies = [RBC basophilic nuclear remnants*(usually removed by spleen macrophages*)] = Pts with autosplenectomy/asplenia(i.e. from Sickle Cell Disease) = will **not** be able to remove [Howell Jolly Bodies]
258
What disease do you see Morulae on peripheral blood smear?
Ehrlichia infection
259
Do NOT confuse Cryoglobulins with Cold IgM hemolysis What are [Cryoglobulins type 2] associated with? - 3
1. Hep C 2. Joint Pain 3. Glomerulonephritis
260
HUS and TTP etx
TTP etx: [A13vM❌(or A13vM inhibition by shiga toxin in HUS)] →uncontrolled vWF chain cleavage → widespread platelet traps/activation → severe thrombocytopenia + [RBC shearing ⼀ microangiopathic hemolytic anemia]) → _FMNRT_ TTP sx ⚠️TTP cp (aside from +Neuro and plasma EXCHANGE tx) = HUS cp. *(TTP Tx = _EXCHANGE_ plasma containing anti-A13vM or deficiency of A13vM for normal plasma)*
261
TTP is associated with what conditions? - 5
1. **CLO**pidogrel 2. ti**CLO**pidine 3. cy**CLO**sporine 4. AIDS *"**CLO**set gay"* 5. {Pregnancy*TTP can onset anytime during [gestation or postpartum pregnancy]*} *"**CLO**set Mom"* *Unlike HUS, [TTP = (neuro sx (confusion/seizure)) / fever / (plasma EXCHANGE tx)]* ## Footnote TTP etx: [A13vM❌(or inhibition by shiga toxin in HUS)] →uncontrolled vWF chain cleavage → widespread platelet traps/activation → thrombocytopenia + microagio hemolytic anemia → _FMNRT_ TTP cp ⚠️TTP cp (aside from +Neuro and plasma EXCHANGE tx) = HUS cp. ⼀ *(TTP Tx = _EXCHANGE_ plasma containing anti-A13vM or deficiency of A13vM for normal plasma)*
262
Tx for Paroxysmal Nocturnal Hemoglobinuria -3
1. Prednisone 2. Bone Marrow Transplant = cure 3. Eculizumab *(inactivates C5 complement)*
263
*"Pt has intense pruritus after a warm shower"* What is the Diagnosis? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ How do you diagnose this?
Polycythemia Vera \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ JAK2 mutation (r/o hypoxia) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *Remember: PV ⬆︎ALL 3 cell lines but places focus on RBC more* *You must exlude Hypoxia as a cause of ⬆︎RBC*
264
Tx for Polycythemia Vera - 3
1. phlebotomy 2. hydroxyurea*Inhibits [Ribonucleotide Reductase] --\> inhibits [DNA thymine] synthesis*→ ⬇︎cell synthesis 3. . [ASA for erythromelalgia (painful red hands from ET or PCV)]
265
When do you treat Essential Thrombocytosis?-2
ONLY when ## Footnote 1. pt\>60 yo with sx OR 2. pt\>60 with [platelets\>1.5million]
266
Tx for Essential Thrombocytosis - 3
1. **HYDROXYUREA***Inhibits [Ribonucleotide Reductase] --\> inhibits [DNA thymine] synthesis* 2. [Anagrelide when RBC is suppressed from Hydroxyurea] 3. [ASA for erythromelalgia (painful red hands from ET or PCV)]
267
DDx for pt presenting with pancytopenia - 7
1. ALL 2. AML 3. [aPL - acute Promyelocytic Leukemia M3 (chromo T15/17)] 4. Aplastic Anemia(Radiation|drugs|Viruses|Fanconi|Idiopathic) 5. [CIPM - Myelofibrosis (dry tap and tear drop cells)] 6. [Myelodysplastic Syndrome (hypercellular bone marrow with ringed sideroblast RBC *with Prussian blue* )] 7. [Hairy Cell Leukemia (dry tap with hypercell bone marrow)]
268
Acute Leukemia will present with signs of ⬜ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Which acute leukemia is associated with [ATRA-all trans retinoic acid]?
**PAN**cytopenia \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [aPL - acute Promyelocytic Leukemia M3 (chromo t1517)] *Dx = smear showing blast --\> flow cytometry for confirmation*
269
Which acute leukemia is intrathecal MTX given to prevent CNS relapse?
**ALL**
270
How is the [LAP-Leukocyte Alkaline Phosphatase] test used for Heme/Onc diagnostics? (5)
❗️[CML(9/22 BCRABL)] ⇪ Granulocyte proliferation ⼀ ❗️[infection (Leukamoid Stress Reaction)] also ⇪ Granulocyte proliferation ⼀ ❗️but [CML Leukemia CA cells] do **NOT** have high levels of *[Leukocyte Alkaline Phosphatase]* and ❗️ ❗️ so… ⇪Granulocytes? Check LAP ⭐[**CML (***LAP elevated?***)INFECTION**] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *CML: Chronic Myelogenous Leukemia*
271
What are the initial therapies for Chronic Myelogenous Leukemia?-3 \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the ultimate cure? *etx: chromo 922 = BCR ABL gene*
tx = [tyrosine kinase inhibitors ( x-tinib)] such as .. 1. imatinib 2. dasatinib 3. nilotinib Cure = Bone marrow transplant (NEVER the first therapy though)
272
What disorder does the [Pseudo⼀Pelger Huet anomaly (*described as ⬜*)] belong to?
✏️[neutrophils with **2-lobe nuclei**(instead of the normal 3-lobe nuclei)] 2/2 [de novo mutations vs exposure(radiation, benzene, chemotherapy)] → dysfunctional hematopoiesis (defective cell maturation) of Myeloid cells(risk of transformation to AML :-( ) ✏️MyeloDysplastic Syndrome
273
Describe peripheral blood smear for [(CLL-SLL)-Chronic Lymphocytic Leukemia - Small Lymphocytic Lymphoma]
proliferation of **normal and mature** (but dysfunctional) B lymphocytes with **smudge cells**
274
What is the Richter phenomenon
conversion of *CLLSLL*--\> [high grade lymphoma (DLBL or Prolymphocytic Lymphoma)] which happens in 5% of *CLLSLL* patients \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *CLLSLL pathology = mature lymphocytes and smudge cells*
275
Hairy Cell Leukemia Tx? ## Footnote *B cells with filamentous projections on smear*
[2CDA\_Cladribineadenosine analog]
276
NonHodgkin Lymphoma and Chronic Lymphocytic Leukemia both involve lymphocyte proliferation What is the major difference
NHL = solid mass (lymph nodes and spleen) CLL = Circulating liquid mass (so use flow cytometry of peripheral blood to diagnose)
277
NonHodgkin Lymphoma Dx? -2
nHL Dx = [**Xbx with CTS ➜ Bbx *for tx***] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [EXCISIONAL bx with CT staging] and [BoneMarrowbx to determine tx] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ nHL dx = HODGKIN LYMPHOMA dx- (except HL *also* requires [⊕ReedSternberg CD15 & CD30 owl eye cells]) = HL Dx = i\⊕CD15 and ⊕CD30[**Xbx with CTS ➜ Bbx *for tx***]
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Tx for [NonHodgkin Lymphoma *with Bsx*] (5)
**CHOPX**
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Tx for [Hodgkin Lymphoma *with B sx*] (4)
**BHOD**
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Tx for [NonHodgkin Lymphoma stage \_\_\_\_\_] *1* *2* *3* *4*
*1* = **R**adiation_local *2* = **R**adiation_local *3* = **CHOPX** *4* = **CHOPX**
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Tx for [Hodgkin Lymphoma stage \_\_\_\_\_] *1* *2* *3* *4*
*1* = **R**adiation_local *2* = **R**adiation_local *3* = **BHOD** *4* = **BHOD**
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Describe the Staging for [NonHodgkin Lymphoma] (5) *“***_T_**all **_B_**rian ***_F_**orgot **_M_**r. **_B_**urkitt's **_D_**og”*
283
Describe the Staging for [Hodgkin Lymphoma] (5)
284
For Hodgkin Lymphoma, what are the determinants for prognosis? (2)
**STAGING= MOST IMPORTANT PGN** > [(Lymphocyte *RICH*) = GOOD Pgn] ## Footnote [(Lymphocyte *mixed*) and (Lymphocyte *poor*))]= poor pgn
285
In Heme/Onc what are the MUGA and nuclear ventriculogram used for?
determines cardiotoxicity for [**HAD**] tx *used in {[Hodgkin Lymphoma] and [NonHodgkin Lymphoma]}* | *🔎HAD = [Hydroxydaunorubicin Adriamycin Doxorubicin]*
286
What are the toxicities for Cisplatin and Carboplatin? - 2
287
What are the toxicities for Vincristine?
288
What are the toxicities for Bleomycin and Bulsulfan?
289
What are the toxicities for Doxorubicin?
290
What are the toxicities for CYclophosphamide?
291
What are the toxicities for 5-FU?
292
What are the toxicities for 6-MP?
293
What are the toxicities for MTX?
294
What are the triggers for DIC - 6
"his DIC **SCABS T**erribly!" 1. **S**epsis 2. **C**A 3. **A**bruptio placenta or Amniotic fluid embolus 4. **B**urns 5. **S**nake bites 6. **T**rauma --\> tissue factor release *DIC activates primary AND secondary coagulation*
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Tx for DIC - 3
1. [FFP*(contains clotting factors but req 2L dose)*] 2. [Cryoprecipitate*(contains clotting factors, vWF, and replaces fibrinogen if FFP doesn't work)*] 3. Platelets if \< 50K | "his DIC **SCABS T**erribly!"
296
Which type of clots are more common with HIT? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ dx for HITT2?-2
Venous \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [Platelet factor 4 Ab on ELISA] or [Serotonin release assay]
297
What abx prophylaxis regimen should pts s/p recent splenectomy receive ?
PCN PO QD x 5 years
298
Bernard Soulier cp - 2
1. Superficial Bleeding out of proportion to the degree of thrombocytopenia 2. GIANT platelets *etx = absent [platelet glycoprotein 1B R] for von willebrand factor to bind to*
299
Why are Bisphosphonates given to CA pts? - 2
stabilizes bony metastatic lesions which 1. prevents CAhypercalcemia 2. prevents CA fx
300
What's the best tx for CA-related anorexia -2
Megestrol*progesterone analogue* ## Footnote >\>\>\> CTS *Marijuana is only useful in HIV anorexia*
301
\*\*HIGH YIELD\*\* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ When is EPO indicated for ESRD pts? What are the side effects of EPO? - 6
Hgb **< 10** (use EPO with hct goal of 35%) ## Footnote \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 1. **HTN** 2. HA 3. Flu-like sx 4. *ischemic strokeEPO given i\ [hgb > 13]* 5. *thrombosisEPO given i\ [hgb > 13]* 6. *⇪all cause mortalityEPO given i\ [hgb > 13]*
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Fanconi anema is an auto recessive disorder with what etx? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ cp?-3
[Fanconi *P-A-N* anemia] = DNA repair defect that → [CP**M**SC❌ (failure/destruction/suppression)) = Aplastic Anemia] → 1. [**Aplastic AnemiaPANcytopenia** marrow failure] → [mucosal bleeding/petechiae], [*NORMO*cytic but hypOproliferative anemia], infection 2. **morphological changes** 3. **growth stunt** ## Footnote *🔎CPMSC = [Common Progenitor **Myeloid** Stem Cell]* *🔎P-A-N = [**P**ancytopenic-**A**plastic-**N**ormocytic]*
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Laboratory results for Chronic Myelogenous Leukemia?-4
1. ⬆︎***ABSOLUTE* BASOPHILIA** 2. ⬆︎⬆︎⬆︎LEUKOCYTOSIS 3. shift tward precursor cells (myelocytes or promyelocytes) 4. ⬇︎Leukocyte Alkaline Phosphatase (LAP) Cure = Bone marrow transplant (NEVER the first therapy though) etx = 922 BCRABL philadelphia chromosome
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Dx for Chronic Myelogenous Leukemia?-3
*"CML has L-L-L-"* [Leukocytosis with **LOW** *LAP*] ## Footnote ❗️ *LAP = *Leukocyte Alkaline Phosphatase = marker of [natural *leukomoid *neutrophil activity]. leukomoid rxn → HIGH*LAP*
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Factor 5 Leiden MOD \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ how does this affect aPTT and PT/INR
AUTO DOM point mutation in Factor 5 gene --\> **RESISTANCE TO PROTEIN C** (which is supposed to inactivate Factor 5). This --\> Hypercoagulability aPTT AND PT/INR may both be normal!
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Dx for Hereditary Spherocytosis - 3
*E5 with Acid* [Eosin 5 maleimide binding flow cytometry] **WITH** [Acidified glycerol lysis test] OR Osmotic fragility test but it has low sensitvity Lab findings = ⬆︎Mean Corpuscular Hgb Concentration
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Hereditary Spherocytosis MOD -3
*[🩸 = "RBC membrane"]** 🟠[🩸*Spectrin*'s inability to anchor [AUTO DOM defective 🩸*Ankyrin*] → [🩸 *bleb formation*] --> splenic macrophages remove but hemolyze/damage RBC in process➜ [**_JAUNDICE_** unconjugated hyperbilirubinemia], \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 🟠 over time, splenic macrophages hypertrophy → [**_SPLENOMEGALY_**]. And [RBC∆(⬇︎MCV with ⇪MCHC, biconcave → spherical) → [High RDW] = ["SPHEROCYTE" RBCs] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 🟠 ULTIMATELY, [SPHEROCYTE RBC ∆ *(small, spherical, no central pallor)*] → [splenic macrophage *_premature removal altogether_* → [*eXtravascular **_hemolytic anemia_** ] Tx = Splenectomy | *🔎MCHC = Mean Cell Hgb Concentration* ## Footnote *_Triad_ = Jaundice, Splenomegaly, Hemolytic Anemia*
308
Which hematological abnormality is Acute Cholecysitis a major complication of?
{[**Hereditary Spherocytosis**_Chronic Hemolysis_] | *Triad = Jaundice, Splenomegaly, Hemolytic Anemia* ## Footnote HS --(*pigmented bilirubin gallstones*)-->[Acute Cholecystitiss]}
309
Although it is a procoagulant, why is lupus anticoagulant called an anticoagulant?
because **ONLY** **in the petri dish,** it causes prolonged aPTT
310
What is Trousseau Syndrome? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What does it indicate?
hypercoagulable disorder --\> recurrent migratory superficical thrombophlebitis at unusual sites (arm, chest) \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Pancreatic Cancer (or sometimes stomach, lung or prostate)
311
A white male presents with megaloblastic anemia, atrophic glossitis, vitiligo and neuro problems... all consistent with Vitamin B12 deficiency What is likely the cause?
GENETIC! Whites of Northern European ancestry naturally develop **Pernicious Anemia** ## Footnote *also, Pernicious Anemia ⬆︎ risk for gastric ADC*
312
Describe etx for Warfarin induced skin necrosis (4)
▶Warfarin inhibits epoxide reductase (which reduces⼀activates Vitamin K(K is responsible for maturing [2, 7, 9, 10, Protein C and S]) → ⬇︎[2,7,9,10, Protein C and S] ▶2,7,9,10 = ⬆︎Clotting ▶[Protein C and S] = anti-Clot = Bleeding. **⭐But since Protein C and S are the first to be affected by warfarin**, allowing 2,7,9,10 to roam freely and ⬆︎Clotting
313
Pernicious Anemia is the most common cause of Vitamin B12 deficiency Pernicious Anemia ⬆︎ risk for developing what type of cancer?
Gastric ADC
314
What disease should you suspect in a pt with Macrocytic anemia and congenital anomalies?
Diamond Blackfan Syndrome (DBS) ## Footnote *intrinsic defect in erythroid progenitor cells --\> ⬆︎apoptosis*
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Type of Cell? ; Diagnosis?
Atypical Reactive CD8 T cells; Infectious Mononucleosis
316
MOD for Hairy cell leukemia? ; How is diagnosis made?
B cell neoplasm that infiltrates bone marrow *→Pancytopenia*, spleen and peripheral blood ; Bone Marrow Biopsy
317
Diagnosis? ; MOD of this disease?
Gaucher ; lysosomal storage disease
318
Describe what Total Iron Binding Capacity (TIBC) measures?
"*_T_ ransferrin _I_n _B_lood _C_alculation*" -TIBC gives a snapshot *(for that that moment in time)* of how much iron the body: -*can* carry (TIBC indicates total capacity [for that moment] the body has to transport iron). ⇪ capacity/TIBC may indicate ⇪ demand) -*wants to carry* (demand)
319
Describe the following values for Thalassemia: MCV Iron Transferrin Saturation TIBC Ferritin
320
Describe the following values for Sideroblastic Anemia: MCV Iron
⬇︎MCV ⬆︎Iron
321
How do you diagnose [CLLSLL]? (2) ## Footnote \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *[CLLSLL-Chronic Lymphocytic Leukemia-Small Lymphocytic Lymphoma]*
1.peripheral blood FLOW CYTOMETRYproliferation of [**normal, mature** (but dysfunctional) B lymphocytes] with 2.peripheral blood smear**smudge cells** * * * ## Footnote *Suspect CLLSLL in any elderly with dramatic leukocytosis primarily made of lymphocytes*
322
Pt has intense pruritus after a warm shower. Polycythemia Vera is diagnosed What is the difference between Phlebotomy and Plasma exchange?
Phlebotomy (tx for PV) removes **cells** while Plasma exchange only removes substances (Antibodies, immune complexes, toxins)
323
What is the most common type of testicular sex cord stromal tumor? ; What does it secrete?-2
Leydig ; Testosterone AND Estrogen ## Footnote *All Solid Testicular Tumor Masses should be treated with Radical Orchiectomy*
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Identify cells ; What 2 diseases are they associated with?
*"Discern {[**EBR**[Echinocyte/Burr=Renal🌀] from **ASL**[Acanthocyte/Spur(ky)=Liver🌀]] ⼀red blood cells}!"* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [**A**canthocyte **S**pur*ky* RBC] = [**L**iver🌀 | Cholesterol❌] | [**A**canthocyte ***S**pur* RBC] are *Spurky = Spiky!* ## Footnote *🌀 = disease*
325
What blood disorder should be suspected in a pt with ⬆︎Mean Corpuscular Hgb Concentration?
Hereditary Spherocytosis
326
Triad for Osler Weber Rendu syndrome
Osler Weber Rendu likes to **EAT** 1. **E**pistaxis recurrently 2. **A**V malformations 3. **T**elangiectasia
327
Which 2 Vitamins are used to treat Homocystinuria?
[Pyridoxine B6] **with** [Folate B9]
328
Tx for iron deficiency anemia?
[ferro**us** sulfate 2+ _ *heme iron*] *”give ***us 2*** more iron”*
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"Pt has intense pruritus after a warm shower" What is the Dx? ; Why does this happen?
Polycythemia Vera \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Heat ⬆︎Basophils (rare but can become AML) --\> ⬆︎Histamine release \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *Remember: PV ⬆︎ALL 3 cell lines but places focus on RBC more*
330
Hairy Cell Leukemia dx?-2 ## Footnote *B cells with filamentous projections on smear*
[TRAP-Tartrate Resistant Acid Phosphatase] or CD11c
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NonHodgkin Lymphoma cp - 2
1. pain**LESS** LAD 2. B sx (Fever, Night sweats, Wt Loss) *Dx = EXCISIONAL bx with staging via CT and BMbx to determine tx* THIS IS THE SAME AS HODGKIN LYMPHOMA - except HD has ReedSternberg owl cells
332
[Acute Intermittent Porphyria] dx \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What factor of a pts hx suggest [Acute Intermittent Porphyria] ?
[(⬆︎Porphobilinogen) in Urine] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Fam hx of similar sx
333
Name the substrate for the [heme synthesis enzyme] \<**U**ROPorph | PCT\>
(**UROPorphyrinogen)** ⬇︎ \<**U**ROPorphoDeCarb | PCT\> “*UPDC PCT”* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *AAP**U**F* *enzyme is associated with [| x]*
334
Name the substrates for the [final heme synthesis enzyme] \<**F**errochelatase | lead tox\> - 3
{[**CoproPorphyrinogen***in cytoplasm* (enters mitochondria)→ **Protoporphyrin***in mitochondria***] + Fe2+ }** ⬇︎ \<**F**errochelatase | lead tox\> ⬇︎ ⭐HEME⭐ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *AAPU**F*** *enzyme is associated with [| x]*
335
Name the substrate for the [heme synthesis enzyme] \<**A**LAD | lead tox\>
(**ALA)** ## Footnote ⬇︎ \<**A**LAD | lead tox\> \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *A**A**PUF* *enzyme is associated with [| x]*
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Name the substrate for the [heme synthesis enzyme] \<**P**orph | AIP\>
(**Porphobilinogen)** ⬇︎ \<**P**orph | AIP\> \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *AA**P**UF* *enzyme is associated with [| x]*
337
Name the substrates for the [heme synthesis enzyme] \<**A**LAS | Sideroblastic\> - 3
[(**Glycine)** + (**Succinyl CoA) + (Pyridoxine B6)]** ## Footnote ⬇︎ \<**A**LAS | Sideroblastic\> \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ ***A**APUF* *enzyme is associated with [| x]*
338
What is the differnece between *Exertional* heat stroke and *NonExertional* heat stroke? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ cp for heat stroke (3)
cp = **HOT** [**H**ead ❌(CNS dysfxn/confusion/weakness/seizures)] [**O**rgan ❌(ARDS/Pulm Edema/NV/Liver Injury/AKI/Rhabdomyolysis/DIC)] **[T**emp core \> 40C (104F)]
339
heat stroke management
cp = **HOT** tx = evaporative /convective cooling with water misters and fan
340
Licorice is commonly found in ⬜ \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ how does it affect BP?
herbal teas;
341
# [Anemia = ⬜] What are the 3 Categories of Anemia?
⬇︎O2 carrying capacity in blood * * * CLDP **LDP** [**L**oss of RBC Blood Volume](Melena, Hematochezia, Ulcers, Fibroids) [**D**estruction of RBC (Hemolytic Anemia)]Intrinsic|Extrinsic in Intravascular|Extravascular [**P**oor production of RBC (⬇︎Erythropoiesis)]
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# [Anemia = ⬜] *Determining cause of Anemia starts with MCV* name DDx for [microcytic anemia\<80 mcv] **(5)**
⬇︎O2 carrying capacity in blood * * * *i*\_**TAILS** \_\_\_\_\_\_\_\_\_\_\_ m*_i_*crocytic
343
# [Anemia = ⬜] *Determining cause of Anemia starts with MCV* name DDx for [Macrocytic anemia \>100 mcv] **(6)**
⬇︎O2 carrying capacity in blood * * * *am*\_**FOB** *an*\_**LAR** \_\_\_\_\_\_\_\_\_\_\_ m*_a_*crocytic
344
# [Anemia = ⬜] *Determining cause of Anemia starts with MCV* Orotic Aciduria causes [⬜anemia] Name major clinical features -4
⬇︎O2 carrying capacity in blood * * * m*_a_*crocytic ; 1. MOD: [auto recessive **defective UMP synthase**] → inability to convert orotic acid to UMP during pyrimidine synthesis. = 2. [macrocytic Megaloblastic anemia (*am\_FOB*)] in kids that ultimately → Failure to Thrive 3. can NOT be cured with *F*olate or *B*12 4. NO hyperammonemia(ornithine transcarbamylase deficiency = ⇪ orotic aciduria WITH hyperammonemia)
345
# [Anemia = ⬜] *Determining cause of Anemia starts with MCV* name DDx for [Normocytic anemia 80-100 mcv] **(15)**
⬇︎O2 carrying capacity in blood ## Footnote [Nn **CATIA**] [Nhi **GHSPCP**] [Nhe **MAIM**]
346
# definition of Anisocytosis
varying sizes of RBC
347
# definition of Poikilocytosis
varying sha**P**es of RBC
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**(image)** is activated by ⬜ and forms ⬜
[*Thrombocyte platelet*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [endothelial tissue injury in 1º hemostasis] ; [*Thrombocyte platelet*] plug after aggregating with other [*Thrombocyte platelets*] and interacting with fibrinogen *⅓ platelets are stored in spleen*
349
Name all the Leukocytes (*Most to Least abundant*) (5)
***N**eutrophils **L**ike **M**aking **E**verything **B**etter* ## Footnote **N**eutrophils **L**ymphocytes **M**onocyte\_macrophages **E**osinophils **B**asophils
350
Describe the breakdown for hematopoietic neoplasia (6)
start with *cell age* * * * *Acute* 1. [Acute\>Lymphoid]= **ALL** 2. [Acute \>Myeloid]= **AML** * * * *Chronic* 1. [Chronic\>Lymphoid\>Chronic Leukemia= **C|H|A**] 2. [Chronic\>Lymphoid\>Lymphoma={**Hodgkin** vs [**NonHodgkin*****"***🆃all 🅱*rian Forgot Mr Burkitt's Dogg" *| * *]} 3. [Chronic\>Lymphoid\>Plasma cell disorders=**MultipleMyeloma** | **Waldenstrommacrogloulinemia**, **MGUS**] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 4. [*Chronic*\>Myeloid\>Myeloproliferative disorders\* (*Constitutively Activated Tyrosine Kinase → Myeloproliferative disorder*) = **1**|**P**|**E**|**C**]([**** Myelofibrosis/CIM] | [**P**olycythemia Vera] | [**E**ssential Thrombocytosis] | [**C**hronic Myelogenous Leukemia]
351
[⇪ band cellsAKA "bandemia" (which are defined as ⬜)] indicates what? (2)
immature neutrophils; { [⇪ **mYeloblast** (*precursor to BENgranulocytes*)] **proliferation**( = bacterial infections or CML)]}
352
What are the causes of Eosinophil activation? (5)
"**NAACP** for *E-Osar-N-Phil*" **N**eoplasia **A**sthma*(limits rxn after mast cell degranulation + produces Histamine)* **A**llergy **C**onnective tissue disease **P**arasites\_invasive*(targets Major Basic Protein = protects from Helminth)*
353
Anaphylaxis (IgE) involves widespread [mast cell & ___ degranulation]. What is a specific marker for mast cell activation?
**basophil** ; Tryptase *Histamine & Heparin are also released*
354
[Burkitt Lymphoma - EBV] is associated with ___ translocation and overexpression of ____ and what 2 symptom forms? Describe the Histo (4)
[8cMyc ➜ 14IG-Heavy Chain]; [cMYC *(and EBV INC B- cell proliferation-->INC translocation risk)*] = [Endemic\_Africa (latent infection w/EBV) → Mandibular lesion] vs [Sporadic\_USA → abd/pelvis lesion] ## Footnote Histo = - "**Starry Sky appearance**" - [Diffuse mid-sized lymphocytes with interspersed macrophages (arrows) - basophilic cytoplasm - and high [Ki-67 fraction-proliferation index]
355
Describe Mast cell (3)
- binds Fc portion of [IgE⼀antigen complex] → cross-links → degranulation(releases histamine, heparin and eosinophil chemotaxis factors) - mediates allergic reaction / [Type 1 hypersensitivity] - [Cromolyn sodium (asthma px)] prevents mast cell degranulation
356
Sickle cell anemia is ___(mode of inheritance) and should be diagnosed with what?
auto recessive; [HgB electrophoresis( will show HgbS with NO_HgbA)] determines carrier status *[Gluta**MATE** --\> Valine @ 6th position of the [β-chain globin]*
357
Hydroxyurea clinical features(3)
1.**Sickle Cell Anemia** (INC Fetal HgbF synthesis) 2.**AML** ⼀(Rapidly ⬇︎WBC) 3.[**CMLwith blast crisis** (**Chronic Myelogenous Leukemia w/blast crisis**)]⼀(Rapidly ⬇︎WBC) * * * *Inhibits [Ribonucleotide Reductase] --\> inhibits [DNA thymine] synthesis*
358
What are the CD Markers for B-lymphocytes? (2)
19 20
359
Why does [Hemolytic Dz of Newborn*(Erythroblastosis Fetalis)* ] occur more in [O**-** Mothers] and less in [A**-** or B**-** Mothers]?
[A**-** or B**-** Mothers] have mostly [anti-Rh Ig**M** antibodies]--Ig**M** *"stays in **M**om"*does NOT cross placenta \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *[TYPE O **-**MOTHERS] HAVE MOSTLY* [anti-Rh Ig**G** antibodies] --Ig**G***"**G**oes across placenta"* will cross placenta! --\> ⇪ HDNEF* | *Ig**G** crosses Placenta*
360
explain how [Vitamin K] works (3)
a. enteric bacteria synthesizes [*oxidized\_inactive* vitamin K] ( this means neonates will not have Vitamin K and need px!) b. [*oxidized\_inactive* vitamin K] is [reduced⼀activated] by [epoxide reductase (inhibited by Warfarin)] → [reduced\_ACTIVATED vitamin K] c. [_**reduced\_**_ACTIVATED vitamin K] acts as cofactor for maturing factors [2,7,9,10,C,S] during synthesis which → [(2,7,9,10)**Pro**\_2ºCoagulation] and [(Protein C,S)**Anti**coagulation*(EARLY Warfarin☠️)*] --------------- ☠️: [(Protein C,S)**Anti**coagulation* are *(EARLY Warfarin Victim☠️)] ➜ 1st to ⬇️ when Warfarin inhibits epoxide reductase = IMPORTANT SINCE ALLOWS FOR 1 WEEK/TEMPORARY UNINHIBITED → [(2,7,9,10)**Pro**\_2ºCoagulation] -➜ = [Warfarin induced skin necrosis]
361
[Protein C and S] are involved with ([**⬜** anti | pro]coagulation) How do they work?
**anti** \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 1-{[endothelial cell thrombin-thrombomodulin complex]} activates [Protein C] 2-[activated Protein C] uses [Protein S] to [cleave ( = inactivate)] 5a and 8a of coagulation cascade
362
What is Ristocetin?
During [Primary hemostasis platelet plugging] vs lab diagnostics, **Ristocetin** activates [vWF (which is already bound to subendothelial collagen exposed from injury)] to bind to [Gp1b\_platelet aDherence R]
363
What is ESR? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ DDx for ⇪ ESR (6)
[Erythrocyte Sedimentation Rate] is used because [Acute-phase reactants (i.e. fibrinogen)] cause RBC to aggregate → higher density than plasma → [⇪ RBC Sedimentation] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 1. **infections** 2. SLE 3. RA 4. temporal arteritis 5. malignant CA 6. Ulcerative Colitis IBD 7. Pregnancy
364
What is ESR? \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ DDx for ⬇︎ ESR (5)
▶[Erythrocyte Sedimentation Rate] = **# of RBC that sediment out of plasma in 1 time unit** ▶certain conditions *[like ⇪ Acute-phase reactants (i.e. ⇪fibrinogen)]* cause RBC to aggregate → [RBC-aggregates] have higher density than plasma →⇪ [# of RBC that sediments out of plasma in 1 time unit] = ⇪ESR \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ ## Footnote *🔲⬇︎ESR*... - polycythemia - Sickle Cell Anemia - CHF - microcytosis - hypOfibrinogenemia
365
Identify finding *(in image) *from pt's peripheral blood smear \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the associated pathology? (2)
*"Discern {[**EBR**[Echinocyte/Burr=Renal🌀] from **ASL**[Acanthocyte/Spur(ky)=Liver🌀]] ⼀red blood cells}!"* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ [**A**canthocyte **S**pur*ky* RBC] = 1. [**L**iver🌀 :Liver Disease] or 2. [Cholesterol❌:abetaLipoproteinemia cholesterol dysregulation] | [**A**canthocyte ***S**pur* RBC] are *Spurky = Spiky!*
366
Identify finding *(in image) *from pt's peripheral blood smear \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the associated pathology? (4)
[Schistocyte helmet *RBC*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 1. DIC 2. TTP 3. HUS 4. [traumatic hemolysis*(i.e. mechanical heart valve )*]
367
Identify finding *(in image) *from pt's peripheral blood smear \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the associated pathology? (3)
[Spherocyte *RBC*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 1. Hereditary Spherocytosis 2. G6PD deficiency 2. autoimmune hemolysis
368
Identify finding *(in image) *from pt's peripheral blood smear \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the associated pathology? (2)
[Macro-ovalocyte (macrocyte) *RBC*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 1. Megaloblastic anemia (am_FOB) 2. Nonmegaloblastic anemia(an_LAR)
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Identify finding *(in image) *from pt's peripheral blood smear \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the associated pathology?
[Ringed sideroblast in *RBC*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Sideroblastic anemia (pathologic excess iron in mitochondria)
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Identify finding *(in image) *from pt's peripheral blood smear \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the associated pathology?
[Teardrop *RBC*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ 1º Myelofibrosis (bone marrow infiltration → forces RBC out of bone marrow → tear shape)
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Identify finding *(in image) *from pt's peripheral blood smear \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the associated pathology?
[Bite *RBC*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ G6PD deficiency (RBC oxidation of hgb sulfhydryl groups < 2/2 loss of G6PD buffer system > → [denatured hgb precipitation (AKA Heinz bodies)]. [Spleen macrophage Phagocytic damage] of these [Heinz bodies] →RBC membrane damage → [Bite *RBC*]
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Identify finding *(in image) *from pt's peripheral blood smear \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the associated pathology?
[howell Jolly bodies in *RBC*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ Spleen❌ *(functional hypOsplenia/asplenia)* (basophilic nuclear remnants in RBC that are usually removed by spleen macrophages)
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Identify finding *(in image) *from pt's peripheral blood smear \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the associated pathology?
[Heinz bodies in *RBC*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ G6PD deficiency (oxidation of hgb sulfhydryl groups → [denatured hgb precipitation (AKA Heinz bodies)]. [Splenic macrophage Phagocytosis] of these [Heinz bodies] →RBC membrane damage → [Bite *RBC*]*(and after so many bites → unstable Spherocyte RBC)*)
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Identify finding *(in image) *from pt's peripheral blood smear \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the associated pathology? (4)
[Basophilic stippling of *RBC*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *"**_Basi_**cally, **AC**id **alcohol** is **Le**-**Thal*** 1. **A**nemia of **C**hronic Disease 2. **alcohol** abuse 3. **Le**ad poisoning 4. **Thal**assemia
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Identify finding *(in image) *from pt's peripheral blood smear \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ What is the associated pathology? (4)
[Target *RBC*] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ *“**HALT**! said the LIVid hunter to his _target_"* 1. **H**bC 2. **A**splenia 3. **L**IVER disease 4. **T**halassemia
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What is [Plummer-Vinson syndrome]? (3)
*“PVS is* *IDA* *as **IEA**”* [iron deficiency anemia (microcytosis, hypOchromia)] that manifest as … ⼀**I**ron deficiency anemia ⼀**E**sophageal webs ⼀**A**trophic glossitis
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Describe [Normal Adult hemoglobin (*Hgb A*)] (3)
[Normal **_A_**dult Hgb *(Hgb A)*] = ## Footnote * * * [4 globin chains: (α2 β2)] = * * * globin:[\< 2 x **α**(chromo 16: 2 alleles per 1 globin) \> + \< 2 x **β**(chromo 11: 1 allele per 1 globin) \>] = * * * alleles: [chromo 16\<αα / αα \> + chromo 11\<β / β \>]
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Sideroblastic Anemia etx (5)
★ → defect in **_ALAS_** of heme synthesis →[*isolated* elevated iron*(specifically in mitochondria)*] → [*Bone Marrow* Ringed sideroblast RBC (that contain iron-laden mitochondria)] ➜ ★ [⇪ iron / ⇪ FerriTin / nl\_TIBC] * * * ★ [X-linked], ★ [acquired (myelodysplastic syndromes)], ★ {reversible (BAIL Out the Copper) with *[Pyridoxine B6 (cofactor for ALAS) tx]*}
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Sickle Cell Disease etx (8)
1. [HgbA gene point mutation causes [Glutamic acid → Valine] at [position 6 of the β chain monomer] = HgbS = 2. [*DICK*/DeOxygenation] of HgbS → [*CAP*-(crystallization aggregative polymerization)] → [HgbSS|SC Sickle Shaped RBC]. 3. Will Un-sickle once O2 is bound again but eventually → RBC Membrane stiffness → a. Chronic Hemolytic Anemia - Intravascular hemolysis 2/2 membrane instability - Extravascular hemolysis 2/2 **red** pulp splenic sequestration of sickles → ultimately causes splenic hypoxia/fibrosis → [auto/functional splenectomy] b. Small Vessel Occlusion by sickles → Vasoocclusive Pain Crisis c. Aplastic Crisis (2/2 Parvo B19 ⬇︎ erythroid precursors) d. [Sickle RBC = ⬇︎1/2 life] → Anemia 4. HgbF is protective
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proposed mechanism for dCCB-associated edema *dCCB=dihydropyridine CCB*
Preferential vasodilation of arteriole–>increased capillary hydrostatic pressure–>increased fluid movement into interstitium
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Which lab is used to follow disease activity in SLE? and why?​
*dana * anti**dsDNA** (indicates and tracks development of lupus nephritis)​
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# ITP etx (4) | [Immune Thrombocytopenic Purpura]
[anti-platelet Ab(against platelet_Gp2b/3A)] start binding to [Platelet R 2b/3A] = forms [Antibody/Platelet complex] *(no obvious cause \> precedes viral/HIV?HCV? infxn)* \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ → splenic macrophages removes [Ab/Platelet complex] from circulation ➜ [_isolated_ thrombocytopenia \<100K] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ - → superficial/mucosal bleeding - [⇪ megakaryocytes on bone marrow biopsy] \_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_\_ -NORMAL SPLEEN -normal clotting factors -normal clotting PT/aPTT ## Footnote *Strongly associated with HIV and HCV*