Med - Heme/Onc Flashcards
1
Q
Dx test for Iron Deficiency Anemia and why
A
FerriTin < 15; measures iron storage and is specifically for IDA
Iron,TIBC,Transferrin can all be low in Anemia of Chronic Dz as well
2
Q
Causes of Normocytic Anemia
NON-Hemolytic (normal or low Retic count) -5
vs
Hemolytic (INC Retic count) - 10
A
3
Q
Is Haptoglobin ⬆︎ or ⬇︎ in Hemolytic Anemia? Why?
A
DECREASED; Liberated Hgb (after RBC hemolysis) BINDS to serum Haptoglobin –> HgbHaptoglobin complex –> Cleared by Liver
Haptoglobin picks up Haphazard hgb
4
Q
Lab markers for Hemolytic Anemia -5
A
- ⬇︎Haptoglobin
- ⬆︎LDH
- ⬆︎UnConjugated bilirubin
- Hgbnuria (Reddish brown urine)
- DAT (Direct Antiglobulin Coombs Test) -detects antibody-mediated hemolysis
5
Q
3 main sources of iron deficiency
A
- ⬇︎ Intake (PO vs GI absorption)
- ⬆︎Output (menorrhagia/hematuria/hemorrhage)
- ⬆︎ Requirement (pregnancy)
6
Q
Most common causes of [Normocytic anemia w/HIGH RDW]-4
A
- Iron deficiency
- B12 Deficiency
- Beta Thalassemia
- hypOthyroidism
7
Q
High RDW typically indicates what etx -4
A
- Mixed deficiency
- Recent Hemorrhage
- Iron deficiency Anemia (late - microcytic)
- Vit B12/Folate deficiency (Macrocytic)
8
Q
[Anemia with Normal RDW] typically indicates ____
A
Thalassemia
9
Q
What about FerriTin makes it non-guaranteed lab value for iron changes?
A
FerriTin is also an acute phase reactant and ⬆︎ with any stress
10
Q
Spinal Cord Compression can be from DJD, Epidural Abscess or Tumor
Which Cancer metastasis are associated with Tumor Spinal Cord Compression? - 5
A
- Prostate
- Renal
- Lung
- Breast
- Multiple Myeloma
11
Q
Which has a longer onset of action: Ibuprofen or Naproxen?
A
Naproxen
12
Q
T or F: Brain Metastasis from NonSOLC is Chemosensitive
A
FALSE!
NonSOLC is NONSensitive
13
Q
CP of Acute Intermittent Porphyria - 3
A
AIP causes PAN
- Psychosis acute onset
- Abd pain acute onset
- Neuropathy acute onset
Fam hx of this is VERY suggestive of AIP
14
Q
Acute Intermittent Porphyria dx ; What factor of a pts hx suggest
A
⬆︎Urine Porphobilinogen ; Fam hx of similar sx
15
Q
When is Cryoprecipitated used?
A
to replace fibrinogen (DIC) - replaces clotting factors in smaller plasma
this is never used first
16
Q
low Ferritin is specific for what type of anemia
A
iron deficiency anemia
Remember that FerriTin is an acute phase reactant
17
Q
What does TIBC measure
A
unbound iron sites on transferrin
i.e. will be elevated in IDA but low in Anemia of Chronic Disease
18
Q
isolated ELEVATED IRON is specific to what type of anemia?
A
Sideroblastic anemia
19
Q
Anemia with normal iron studies is specific for what type of anemia?
A
Thalassemia (except 3 gene deletion alpha thalassemia)
Dx = Hgb electrophoresis with genetic studies if alpha thalassemia
20
Q
Tx for iron deficiency anemia? ; sideroblastic anemia
A
ferrous sulfate 2+ ; Pyridoxine B6
21
Q
What is Cooley Anemia? ; tx?
A
Beta thalassemia MAJOR (BOTH Beta proteins on chromo 11 are underproduced) –> SEVERE ANEMIA
Tx = chronic transfusion with deferadirox iron chelator
22
Q
Causes of Vitamin B12 deficiency - 6
A
- PERNICIOUS ANEMIA = MOST COMMON CAUSE
- Vegan/Vegetarian
- Blind loop syndrome (Gastrectomy or RYGB)
- Diphyllobothrium latum
- Pancreatic Insufficiency
- Terminal iLeum damage (Crohns)
23
Q
how do you differentiate Vitamin B12 deficiency from Folate deficiency
A
Vitamin B12 isomerizes methymalonyl coA in the spinal cord myelin —> succinyl coA.
Without it –> suBACute combined degeneration
usually manifest as peripheral neuropathy
24
Q
Causes of Folate deficiency - 4
A
- Goat Milk diet (has no folate)
- Psoriasis
- Phenytoin
- Sulfa drugs
25
Chronic hemolysis is associated with what type of gallstones?
**Pigmented** bilirubin gallstones
26
In sickle cell, what causes vasoocclusive crisis -4
**DICK**
1. **D**ehydration
2. **I**nfection
3. **C**old temp
4. **K**ant breathe (HYPOXIA)
27
Sickle Cell Disease dx
Initially: Peripheral Smear (since SCtrait won't have sickled cells)
## Footnote
**Confirmation: Hgb Electrophoresis**
28
What type of figures are found on smear in Sickle Cell Disease
Howell Jolly Bodies (left over nuclear material in pts who don't have a spleen to remove it)
29
What disease do you see Bite cells?
G6PD deficiency
30
What disease do you see Morulae on peripheral blood smear?
Ehrlichia infection
31
Tx for Sickle Cell Pain Crisis - 4
1. **HOA** (**H**ydration/**O**xygen/**A**nalgesia)
2. Ceftriaxone if fever/WBC present
3. Hydroxyurea ⬆︎HbF
4. Transfusion if Acute Chest Syndrome, Priapism, Stroke, Retinal infarction
32
How does ParvoB19 affect Sickle Cell Disease pts
parvoB19 causes Aplastic Crisis which freezes any further reticulocyte development from marrow. SC pts usually always have high reticuloctye counts but when hit with parvoB19, they suddenly have a drop
33
Tx for Hereditary Spherocytosis - 2
1. Folic acid chronically
2. Splenectomy (stops hemolysis)
34
MOD for Warm IgG hemolysis
Autoimmune Ab **in the spleen or liver** bind to RBC and remove small amounts of the membrane --\> microspherocytes
35
Tx for Warm IgG Hemolysis - 4
1st = **\*\* CTS initially \*\*** --\> IVIG
2nd = Splenectomy ---\> Riuximab
36
What are the triggers of Cold IgM hemolysis - 3
**cold** weather is **M****MM**iserable
1. **M**ononucleosis EBV
2. **M**ycoplasma PNA
3. **M**acroglobulinemia Waldenstrom
*occurs in colder (peripheral) parts of body and resolve with warming up body*
37
Which tx for Warm IgG hemolysis can NOT be given to Cold IgM hemolysis - 2
CTS
SPLENECTOMY
38
Do NOT confuse Cryoglobulins with Cold IgM hemolysis
What are Cryoglobulins associated with? - 3
1. Hep C
2. Joint Pain
3. Glomerulonephritis
39
List the 4 main characteristics of G6PD deficiency
**Stress** makes me eat **bites** of **fava beans** with **Heinz** ketchup
1. oxidant **Stress** on RBC from lack of glutahione reductase --\> hemolysis and SUDDEN BACK PAIN
2. **B****ite** cells
3. Induced by **fava beans**, macrobid, sulfa, primaquine, infection
4. **Heinz** bodies (RBC inclusions seen after crystal violet staining)
*Almost always in Black/Mediterranean Men with sudden Anemia*
40
HUS and TTP MOD
TTP WILL HAVE MORE NEURO SX and it's tx = plasma **exchange**
*otherwise, HUS and TTP present the same*
41
TTP is associated with what conditions? - 4
1. Clopidogrel
2. Ticlopidine
3. Cyclosporine
4. AIDS
## Footnote
*Unlike HUS, TTP will have neuro sx (confusion/seizures) in addition to Fever*
42
When is it ok to give Platelet transfusion to HUS/TTP?
**NEVER!!!**
## Footnote
TTP may receive Plasma exchange for tx
43
MOD for Paroxysmal Nocturnal Hemoglobinuria
[CD55/59 Decay Accelerating factor] (responsible for deactivating complement) of the RBC, WBC and platelets is produced less
so...
nocturnal respiratory acidosis (shallow breathing during sleep) --\> complement activation --\> complement binds to RBC, WBC and platelets --\> nocturnal hgbnuria/⬆︎infection/thrombosis
44
What is the most common cause of death in Paroxymal Nocturnal Hemoglobinuria
Thrombosis (Mesenteric or Hepatic veins)
45
Tx for Paroxysmal Nocturnal Hemoglobinuria
1. CTS prednisone
2. Bone Marrow Transplant = cure
3. Eculizumab inactivates C5 complement
46
Treatment for Aplastic Anemia is Bone Marrow Transplant
At what age does this become unavailable? ; what are the alternatives then?-2
\>50 yo ; [AntiThymocyte Globulin and Cyclosporine]
47
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
## Footnote
* You must exlude Hypoxia as a cause of ⬆︎RBC*
* Dx = JAK2 mutation*
* Remember: PV ⬆︎ALL 3 cell lines but places focus on RBC more*
48
Tx for Polycythemia Vera - 2
1. phlebotomy
2. hydroxyurea
49
When do you treat Essential Thrombocytosis?-2
ONLY when pt\>60 yo and [sx or platelets are \>1.5million]
50
Tx for Essential Thrombocytosis - 3
1. **HYDROXYUREA**
2. Anagrelide when RBC is suppressed from Hydroxyurea
3. ASA for erythromelagia (painful red hands from ET)
51
Aplastic Anemia and Myelofibrosis both present as Pancytopenias
What are 3 discerning factors?
1. Myelofibrosis occurs in older people
2. Myelo = teardrop shaped cells from cells struggling to get out of **fibrosed bone marrow**
3. Myelo tx = [Thalidomide and Lenalidomide] = bone marrow production stimulators
52
Acute Leukemia will present with signs of \_\_\_\_\_
Which acute leukemia is associated with DIC?
**PAN**cytopenia ; promyelocytic M3 (chromo 1517)
## Footnote
*Dx = smear showing blast --\> flow cytometry for confirmation*
53
Acute Leukemia will present with signs of \_\_\_\_\_
Which acute leukemia is associated with Auer rods (eosinophilic inclusions)?
**PAN**cytopenia ; promyelocytic M3 (chromo 1517)
## Footnote
*Dx = smear showing blast --\> flow cytometry for confirmation*
54
Acute Leukemia will present with signs of \_\_\_\_\_
Which acute leukemia is associated with Myeloperoxidase?
**PAN**cytopenia ; Myelocytic
## Footnote
*Dx = smear showing blast --\> flow cytometry for confirmation*
55
DDx for pt presenting with pancytopenia - 5
1. Acute Leukemia
2. Aplastic Anemia
3. Myelofibrosis (dry tap and tear drop cells)
4. Myelodysplastic Syndrome (hypercellular bone marrow with ringed sideroblast with Prussian blue )
5. Hairy Cell Leukemia (dry tap with hypercell bone marrow)
56
Acute Leukemia will present with signs of \_\_\_\_\_
Which acute leukemia is associated with ATRA-all trans retinoic acid?
**PAN**cytopenia ; promyelocytic M3 (chromo 1517)
## Footnote
*Dx = smear showing blast --\> flow cytometry for confirmation*
57
Which acute leukemia is intrathecal MTX given to prevent relapse?
**ALL** is treated with intrathecal MTX to prevent CNS releapse
58
How is the [LAP-Leukocyte Alkaline Phosphatase] test used for Heme/Onc diagnostics?
Leukemic cells do NOT have high levels of Alkaline Phosphatase so in CML, LAP will be low
if LAP is high = leukemoid rxn (stress rxn)
59
Other than [LAP-Leukocyte Alkaline Phosphatase] test, what else can be used to diagnose CML?
[BCR-ABL 922] PCR
60
Out of the Myeloproliferative disorders, which has greatest potential for transformation into Acute Leukemia BLAST CRISIS?
CML
61
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 such as ..
1. imatinib
2. dasatinib
3. nilotinib
Cure = Bone marrow transplant (NEVER the first therapy though)
62
In pt presenting with acute leukostasis reaction, what is the FIRST important thing to do for them?
Leukaphresis (establish dx AFTER!)
63
Myelodysplastic Syndrome is a PreLeukemic disorder
Why do most pts never actually develop AML from Myelodysplastic Syndrome?
They die of infection or bleeding FIRST
## Footnote
MDS causes pancytopenia despite hypercellular bone marrow
64
Which chromosomal abnormality is Myelodysplastic Syndrome associated with?
5q deletion (these pts have better pgn)
65
What disorder does Pelger Huet cells belong to?
## Footnote
*Cell with Bilobed nucleus*
MyeloDysplastic Syndrome
66
Describe peripheral blood smear for CLL-Chronic Lymphocytic Leukemia
proliferation of **normal and mature** (but dysfunctional) B lymphocytes with **smudge cells**
67
What is the Richter phenomenon
conversion of Chronic Lymphocytic Leukemia --\> high grade lymphoma which happens in 5% of patients
## Footnote
*CLL = mature lymphocytes and smudge cells*
68
What px should be given in Chronic Lymphocytic Leukemia
PCP
69
Tx for Chronic Lymphocytic Leukemia - 2
Stage
[0: ⬆︎WBC] and [1: LAD] = no tx
[3: HepatoSplenomegaly], [4: Anemia], [5:Thrombocytopenia] = Fludarabine and Rituximab(if available)
70
Hairy Cell Leukemia dx?-2 ; Tx?
## Footnote
*B cells with filamentous projections on smear*
Dx = [TRAP-Tartrate Resistant Acid Phosphatase] or CD11c
Tx = Cladribine
71
Non-Hodgkin 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)
72
NonHodgkin Lymphoma cp - 2 ; Dx?
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
73
Tx for NonHodgkin Lymphoma with Bsx or ≥ Stage 3 -5
74
Tx for NonHodgkin Lymphoma stage 1A and 2A
75
For Hodgkin Lymphoma, what are the determinants for prognosis?
Lymphocyte Predominant = GOOD Pgn
76
Tx for Hodgkin Lymphoma with Bsx or ≥Stage 3 - 4
77
In Heme/Onc what are the MUGA and nuclear ventriculogram used for?
Determine cardiotoxicity for Adriamycin/Doxorubicin tx used for Hodgkin Lymphoma
78
What are the toxicities for Cisplatin and Carboplatin? - 2
79
What are the toxicities for Vincristine?
80
What are the toxicities for Bleomycin and Bulsulfan?
81
What are the toxicities for Doxorubicin?
82
What are the toxicities for Trastuzumab?
83
What are the toxicities for CYclophosphamide?
84
What are the toxicities for 5-FU?
85
What are the toxicities for 6-MP?
86
What are the toxicities for MTX?
87
Multiple Myeloma MOD
abnml proliferation of plasma cells that produce useless immunoglobulin (IgG , IgA) --\> [Osteoclast Activating Factor] and **CRABB** (hyper**C**alcemia, **R**enal failure, **A**nemia, **B**one lytic lesions, **B**ack pain)
When MM produces IgM = Waldenstrom Macroglobulinemia
88
cp for Multiple Myeloma - 5
**CRABB**
(hyper**C**alcemia, **R**enal failure, **A**nemia, **B**one lytic lesions, **B**ack pain)
**Hypercalcemia** --\> Bone lytic lesions, CONSTIPATION, Depression
**Renal failure** from Bence Jones proteinuria and Immunoglobulins --\> DEATH
**Infection** --\> DEATH
Osteoclast Activating Factor -\> Hypercalcemia and Bone fxs
89
Multiple Myeloma dx - 5
1. Xray showing osteo**LYTIC** fx lesions from hypercalcemia
2. Serum Protein Electrophoresis showing IgG or IgA monoclonal "M" Spike (can also be seen in MGUS)
3. Bence Jones proteinuria via urine immunoelectrophoresis only
4. Rouleaux on smear
5. **\*\*\*Bone marrow bx showing \> 10% plasma cells\*\*\* = CONFIRMATION**
90
Multiple Myeloma tx - 4
1. [Bortezomib proteasome inhibitor] and/or LD
2. [LD- Lenalidomide + Dexamethoasone] --\> Bone Marrow Transplant if \< 70 yo
91
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 transmorts to myleoma 1%/year
92
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
93
Waldenstrom Macroglobulinemia tx - 3
1. **PLASMAPHERESIS initially**
2. Chlorambucil + Prednisone = long term OR
3. Fludarabine + Prednisone = long term
94
ITP cp - 2
1. **isolated** thrombocytopenia +/- megakaryocytes on smear (typically after infection)
2. Normal spleen
Strongly associated with HIV and HepC
95
ITP tx - 5
Strongly associated with HIV and HepC
96
What's the most common inherited bleeding disorder
AUTO DOM Von Willebrand Disease
## Footnote
*worst after using ASA*, *detected via Ristocetin cofactor assay*
97
tx for Von Willebrand disease - 3
DDAVP desmopressin --(refractory)--\> Factor 8 or vWF concentrate
## Footnote
(DDAVP releases subendothelial stores of vWF)
98
What type of bleeding is seen with Factor 11 Deficiency
USUALLY NO INCREASED BLEEDING
99
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*
100
Tx for DIC - 3
1. Platelets if \< 50K
2. FFP (clotting factors)
3. Cryoprecipitate (replaces fibrinogen if FFP doesn't work)
101
Which type of clots are more common with HIT? ; dx for HIT?-2
Venous ; Platelet factor 4 Ab on ELISA or Serotonin release assay
102
Lupus Anticoagulant dx
Russel Viper Venom test
103
What is the most common complication for pts with Sickle Cell Trait?
Painless hematuria
## Footnote
*Sickle cell is auto recessive*
104
What prophylaxis should pts who've recently had a splenectomy receive afterward?
Daily PO PCN x 5 years
105
Bernard Soulier cp - 2
1. Superficial Bleeding out of proportion to the degree of thrombocytopenia
2. GIANT platelets
## Footnote
*etx = absent platelet glycoprotein 1B-9-5 Receptor for von willebrand factor*
106
In pts with Factor defects (secondary coagulation dysfunction), what is the long term of blood sitting in their joints?
Hemophilic arthropathy
iron/hemosiderin desposition --\> synovitis and fibrosis of that joint --\> chronic worsening joint pain and swelling
107
The vast majority of Head and Neck CA (i.e. submandibular uL hard non-tender LAD) are what type of CA?
SQC
108
Why are Bisphosphonates given to CA pts? - 2
stabilizes bony metastatic lesions which
1. prevents hypercalcemia of CA
2. prevents fx
109
What is the most common cause of Folate B9 deficiency in the U.S.?
EtOH
110
111
What are the major electrolyte changes in Tumor Lysis Syndrome - 3
cytotoxic chemotherapy --\>
1. ⬆︎K+ --\> cardiac arrhythmias
2. ⬆︎uric acid (give px allopurinol and IVF)
3. ⬆︎Phosphate (will bind and ⬇︎ serum Ca+)
112
GVHD-Graft Versus Host Disease is common after \_\_\_\_\_
It involves Graft/Donor __ cells attacking which 3 parts of the body?
Bone marrow transplant ; T ;
1. Skin
2. GI
3. Liver
*This occurs because Graft/Donor T-cells recognize major and minor HLA antigens of the Host*
113
What's the best tx for CA-related anorexia -2
Megestrol progesterone analogue \>\>\> CTS
## Footnote
*Marijuana is only useful in HIV anorexia*
114
\*\*HIGH YIELD\*\*
When is EPO indicated for ESRD pts?
What are the side effects of EPO? - 3
Hgb\<10 (use EPO with hct goal of 35%)
1. **HTN**
2. HA
3. Flu-like sx
115
How can you differentiate between Bone Marrow Infiltration and Bone Marrow Aplasia using the spleen?
Splenomegaly only occurs in Bone Marrow Infiltration
116
Describe Splenic Sequestration
complication of Sickle Cell Disease in which a pooling of RBC AND PLATELETS within the spleen --\> splenomegaly, pallor and **thrombocytopenia**
117
Sickle Cell Disease pts are at risk of having Aplastic Crisis
What is the difference between Aplastic Crisis and Aplastic Anemia?
Aplastic Anemia is a/w **PAN**cytopenia and can be congenital (fanconi, drug-induced, autoimmune)
118
Sickle Cell Disease pts are at risk of having Aplastic Crisis
Clinical Presentation of this? ; What's the most common cause of this?
1. SUDDEN drop in Hgb with
2. Reticulocyte \< 1%
3. NO Hepatosplenomegaly
Parvo B19!
119
Fanconi anema is an auto recessive disorder that causes \_\_\_\_(micro/normo/macro) anemia
cp?-3
Fanconi MACROcytic anemia
1. PANcytopenia marrow failure
2. morphological changes
3. growth stunt
120
Laboratory results for Chronic Myelogenous Leukemia?-3
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
121
Dx for Chronic Myelogenous Leukemia?-3
**LOW** Leukocyte Alkaline Phosphatase score (marker of neutrophil activity and differentiates from leukomoid rxn)
122
lab values for Acute Lymphoblastic Leukemia - 3
1. **\> 25% Lymphoblast from bone marrow bx (THIS IS HOW YOU DIAGNOSE)**
2. TdT positive (TdT is only expressed by preB and preT lymphocytes)
3. PAS positive
cp: LAD, hepatosplenomegaly, thrombocytopenia
123
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)
124
Describe the Transfusion Reaction timeline - 4
125
What is the px for Febrile nonhemolytic reactions after transfusion
Leukoreduction of donor blood
## Footnote
*this will also ⬇︎ risk of HLA alloimmunization and CMV transmission*
126
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 inactive Factor 5). This --\> Hypercoagulability
aPTT AND PT/INR may both be normal!
127
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
128
why are sickle cell patients (both trait and disease) at ⬆︎for benign nocturia
**HYPOSTHENURIA** ; This is when sickles obstruct the vasa rectae of the inner medulla --\> inability to concentrate urine
Happens in Sickle Cell pts
129
Autoimmune Hemolytic Anemia and Hereditary Spherocytosis BOTH can cause extravascular hemolytic anemia
How can you discern the two? - 2
Fam hx and Coombs test
AIHA = **Positive** Coombs test (negative fam hx)
Hereditary Spherocytosis = **NEG** Coombs test (positive fam hx)
130
G6PD deficiency MOD
**Stress** makes me eat **bites** of **fava beans** with **Heinz** ketchup
Triggers --\> hemolysis
*Almost always in Black/Mediterranean Men with sudden Anemia*
131
Hereditary Spherocytosis MOD
Defect in RBC membrane (ankyrin, band and spectrin) --\> spleen removing the defective parts lil by lil as RBC past which eventually --\> spherocytes which have High mean cell hgb concentration and RBC distribution width
132
In Transfusion Reactions both Anaphylaxis and Acute Hemolytic Reactions can occur within minutes
How do you differeniate the two?
Anaphylaxis (especially if pt is IgA deficient) = Respiratory Distress
Acute Hemolytic Reaction = Flank pain with F/C
133
Which type of clots are Heparin Induced Thrombocytopenia pts at risk for, Venous or Arterial?
BOTH
134
A pt coming in with GI distress secondary to EColi should be worked up for what heme condition
Hemolytic Uremic Syndrome HUS HAT
## Footnote
HUS HAT from EColi O157 H7 shiga toxin
**H**emolytic Anemia
**A**cute renal failure
**T**hrombocytopenia
135
What are the 3 examples of Microangiopathic Hemolytic Anemia
HAT (HemolyticAnemia/AcuteRenalFailure/Thrombocytopenia)
1. DIC
2. HUS
3. TTP (will have more neuro signs)
136
What do anabolic steroids affects hematologic lab values? - 3
1. ⬆︎RBCs
2. Hepatotoxic
3. Dyslipidemia
137
Dx
Thalassemia
## Footnote
*image shows teardrop cells (Thalassemia also has Target cells)*
138
what's the most common cause of anemia in premature infants?
Anemia of Prematurity
## Footnote
etx: ⬇︎EPO, shortened RBC life
139
TTP tx
TTP will have more neuro sx and it's tx = plasma **exchange**
140
DIC tx
Cryoprecipitate
## Footnote
(contains clotting factors, fibrinogen and vWF)
141
Hereditary Spherocytosis MOD
Defect in RBC membrane (ankyrin, band and spectrin) --\> spleen removing the defective parts lil by lil as RBC past which eventually --\> spherocytes eventually --\> hemolysis
## Footnote
*Triad = Splenomegaly, Jaundice, Hemolytic Anemia*
142
Which hematological abnormality is Acute Cholecysitis a major complication of?
Hereditary Spherocytosis (from pigmented gallstones)
## Footnote
*Triad = Splenomegaly, Jaundice, Hemolytic Anemia*
143
Although it is a procoagulant, why is lupus anticoagulant called an anticoagulant?
because **ONLY** **in the petri dish,** it causes prolonged aPTT
144
Describe the peripheral blood smear for Chronic Myeloid Leukemia
leukocytosis **with shift toward precursor cells (promyelocytes, myelocytes)**
Cure = Bone marrow transplant (NEVER the first therapy though)
145
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)
146
When is empiric anticoagulation ok for DVT/PE?
ONLY when pt has sx suggesting PE is present . If only DVT sx are present, confirm with US first before anticaogulation
147
How long does it take a vegan to develop macrocytic anemia secondary to vitamin B12 deficiency
≥4 years
148
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*
149
Describe etx for Warfarin induced skin necrosis
Warfarin ⬇︎2, 7, 9, 10, Protein C and S
27910 = ⬆︎Clotting
[Protein C and S] = anti-Clot = Bleeding. But Protein C and S are the first to be affected by warfarin, allowing 27910 to rome freely and ⬆︎Clotting
150
Pernicious Anemia is the most common cause of Vitamin B12 deficiency
Pernicious Anemia ⬆︎ risk for developing what type of cancer?
Gastric ADC
151
What is the mangement for when a pt develops Heparin Induced Thrombocytopenia (HIT) - 3
1st: **STOP HEPARIN OR ENOXPARIN**
2nd: initiate alternative (dabigatran, fondaparinux)
3rd: initiate Warfarin once platelet is \> 150K
152
An elderly pt has minor trauma and develops ecchymoses
What should be first on the DDx for this particular pt?
Senile Purpura (not heme related)
Age-related ⬇︎of elastic fibers in perivascular connective tissue --\> purpura
153
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*
154
What gene abnormality causes Chronic Myelogenous Leukemia?
chromo 922 which forms BCR ABL gene
## Footnote
Cure = Bone marrow transplant (NEVER the first therapy though)
155
Type of Cell? ; Diagnosis?
Atypical Reactive CD8 T cells; Infectious Mononucleosis
156
MOD for Hairy cell leukemia? ; How is diagnosis made?
B cell neoplasm that infiltrates bone marrow, spleen and peripheral blood; Bone Marrow Biopsy
157
Diagnosis? ; MOD of this disease?
Gaucher ; lysosomal storage disease
158
Describe what Transferrin measures?
The amount of iron being transferred in the blood
159
Describe what Total Iron Binding Capacity (TIBC) measures?
The Capacity of Iron transferrin can actually carry
160
Describe the following values for Iron Deficiency Anemia:
MCV
Iron
Transferrin
TIBC
Ferritin
161
Describe the following values for Thalassemia:
MCV
Iron
Transferrin
TIBC
Ferritin
162
Describe the following values for Anemia of Chronic Disease:
MCV
Iron
Transferrin
TIBC
Ferritin
163
Describe the following values for Sideroblastic Anemia:
MCV
Iron
⬇︎MCV
⬆︎Iron
164
How do you diagnose CLL-Chronic Lymphocytic Leukemia?
## Footnote
*Suspect this in any elderly with dramatic leukocytosis primarily made of lymphocytes*
Flow Cytometry of peripheral blood
## Footnote
proliferation of **normal and mature** (but dysfunctional) B lymphocytes with **smudge cells**
165
An elderly pt who presents with dramatic leukocytosis primarily made of lymphocytes should always make you suspicious of what disease?
Chronic Lymphocytic Leukemia (CLL SLL)
proliferation of **normal and mature** (but dysfunctional) B lymphocytes with **smudge cells**
166
MOD for Pica
iron deficiency anemia --\> desire to eat **ice, clay, dirt, paper**
167
A pt is newly diagnosed with Head and Neck Squamous cell carincoma
What is the next best step in their diagnostic process?
PANendoscopy (esophagoscopy, bronchoscopy, laryngoscopy)
168
Pt has intense pruritus after a warm shower
What is the Dx? ; What is the tx for this?
Polycythemia Vera ; Phlebotomy
## Footnote
* You must exlude Hypoxia as a cause of ⬆︎RBC*
* Dx = JAK2 mutation*
* Remember: PV ⬆︎ALL 3 cell lines but places focus on RBC more*
169
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)
170
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*
171
A pt has been diagnosed with a Solid Testicular Tumor Mass via Ultrasound
Tx? -2
**Radical Orchiectomy** --\> additional chemoradiation/surgery based on cell type
172
Identify cells ; What disease are they associated with?
Spur cell acanthocytes ; Liver disease
173
What blood disorder should be suspected in a pt with ⬆︎Mean Corpuscular Hgb Concentration?
Hereditary Spherocytosis
174
cp for Immune Thrombocytopenia ; tx?-3
isolated thrombocytopenia after an infection (usually in a child) ;
1. usually self limited to 6 months = observation
2. IVIG **if bleeding present** OR
3. CTS **if bleeding present**
175
What type of gallstones are pts with Hereditary Spherocytosis at risk for?
Pigmented Bilirubin Gallstones
in Hereditary Spherotycosis, RBC membrane defect can cause intravascular hemolysis
176
Triad for Osler Weber Rendu syndrome
Osler Weber like to **EAT**
1. **E**pistaxis recurrently
2. **A**V malformations
3. **T**elancietasia
177
Which 2 Vitamins are used to treat Homocystinuria?
Pyridoxine B6 **with** Folate B9
