Heme - Onc Flashcards
1
Q
Extramedullary Hematopoiesis is most commonly caused by what?
A
[Beta-thalassemia] = [Chronic Hemolytic Anemia]
2
Q
Explain the 2 steps of the Schilling Test
A
1st: [Oral Vit B12] is given with [IV Vit B12] –> Excess [Vit B 12]. Urine is collected and should show [HIGH urinary excretion] of [Vit B12]= indicates [Vit B12] was [intestinally absorbed] properly into the circulatory system
(low urinary excretion = possible malabsorption problem and rules out dietary deficiency)
2nd: Repeat, but do with [Intrinsic Factor]
(If it corrects = IF deficiency was the issue)
( If doesn’t correct = TRUE MALABSORPTION PROBLEM)
3
Q
Name 4 Common causes of Vitamin B12 Deficiency
A
- GIP [Gastritis / Ilial Resection / Pancreatitis]
- Fish Tapeworm
- Atrophic Gastritis –> Destruction of [Parietal Cells that produce IF] –> DEC [Intrinsic Factor]
- Strict Vegetarians (Proteins contain Vit B12)
4
Q
A: List the Dx Criteria for SLE - Systemic Lupus Erythematosus
B: How many of these criteria must be met for diagnosis?
A
BUBA’S a KAPPA Dude
- Butterfly Malor Rash
- Ulcers (Oral vs. Nasal)
- Brain Dz (seizures / psychosis)
- Arthritis (can tx w/anti-malarial)
- Serositis (pericarditis / pleurisy / peritonitis)
- Kidney Dz (Lupus Membranous Nephropathy) vs. (Lupus Nephritis)
- Anti-nuclear Antibodies
- Pancytopenia (includes compliment)
- Photosensitivity
- Autoantibodies (dsDNA vs. Smith vs. Phospholipid(False positive VLDR) )
- Discoid Rash anywhere on body
B: At least 4
5
Q
Where does Extramedullary Hematopoiesis for this condition occur? (2)
A
Liver and Spleen
6
Q
Classic presentation (3) for [Peau d’Orange (inflammatory Breast CA)]
and MOD
A
[Erythematous / itchy / indurated (orange peel) Rash] caused by
CA spreading to dermal lymphatic space –> obstructing lymphatic drainage
7
Q
Key Features of [Ductal Carcinoma In Situ] (3)
Invasive or NonInvasive?
A
8
Q
Key Features of [Paget Breast CA] (2)
Invasive or NonInvasive?
A
9
Q
Key Features of [Ductal Carcinoma] (2)
Invasive or NonInvasive?
A
10
Q
Key Features of [Lobular Carcinoma] (2)
Invasive or NonInvasive?
A
11
Q
The HER2Neu oncogene encodes for a ______ glycoprotein that has intracellular _____ activity and is in the ____ family. This ultimately does what?
A
The HER2Neu oncogene encodes for a [185kD transmembrane] glycoprotein that has intracellular Tyrosine Kinase activity and is in the EGFR family. Ultimately accelerates cell proliferation.
12
Q
How does Warfarin cause Skin Necrosis? Which demographic? Onset?
A
Warfarin inhibits Protein C and S (natural anticoagulants) –> Skin Necrosis.
People deficient in these are at INC risk
Onset = first few days of warfarin therapy
13
Q
Manifestations of Atrophic Gastritis (3)
A
- hypOchlorhydria
- DEC IF production –> VitB12 Deficiency
- INC methylmalonic acid
14
Q
[Identify and Describe Arrows] and then Dz
A
AML (Acute Myeloid Leukemia)
[Blast = immature myeloid precursors that replace normal bone marrow]–> Pancytopenia
Auer rods = [Peroxidase linear puple inclusions]
15
Q
Von Willebrand Dz
A: Platelet Count
B: PT time
C: aPTT time
D: Fibrinogen level
E: Describe Peripheral Blood Smear
A
16
Q
[IITP - Immune Idiopathic Thrombocytopenic Purpura]
A: Platelet Count
B: PT time
C: aPTT time
D: Fibrinogen level
E: Describe Peripheral Blood Smear
A
17
Q
TTP-HUS
A: Platelet Count
B: PT time
C: aPTT time
D: Fibrinogen level
E: Describe Peripheral Blood Smear
A
18
Q
DIC
A: Platelet Count
B: PT time
C: aPTT time
D: Fibrinogen level
E: Describe Peripheral Blood Smear
A
19
Q
[IITP - Immune Idiopathic Thrombocytopenic Purpura]
MOD
A
Autoantibodies destroy platelets –> Isolated Thrombocytopenia w/ NO splenomegaly
20
Q
Clinical Sx for [Systemic Lupus Erythematosus] (8)
A
- Constitutional (Fever / Fatigue / Wt. Loss)
- Arthritis -Symmetric & Migratory
- [Malor Butterfly Rash]
- Photosensitivity
- Serositits (pericarditis & peritonitits)
- Thromboembolism (due to antiphospholipid Ab)
- Seizures & Cognition DEC
- [Lupus Nephritis = PrOteinuria + INC Creatinine (Type 3) Hypersensitivity]
21
Q
Labs for [Systemic Lupus Erythematosus] (4)
A
- Pancytopenia (Hemolytic Anemia Type 2 Hypersensitivity) / Thrombocytopenia / Leukopenia)
- hypOcomplent (C3 & C4)
- [ANA Ab (sensitive)]
- [Anti-dsDNA and SM (specific)]
22
Q
Anaplastic tumors are ____ and bear no resemblance to the original tissue. They often contain _____ cells and _____
A
Anaplastic tumors are Undifferentiated and bear no resemblance to the orginial tissue. They often contain [Giant Multinucleated Tumor cells] and Pleomorphism
23
Q
Name a common and serious complication of [Chronic Hemolytic Anemia] with Blood transfusion.
How is this treated?
A
Hemosiderosis (Iron overload) - typically manifested as Hemosiderin accumulation in Kupffer macrophages.
Tx = Iron Chelation
24
Q
Name the CA that cause [OsteoBLastic Bone Metastases] (3)
A
Prostate
SOLC
Hodgkin Lymphoma
25
Name the CA that cause BOTH [Osteo**BLastic** and Osteo**lytic** Bone Metastases] (2)
GI
Breast
26
Name the CA that cause [Osteo**lytic** Bone Metastases] (5)
27
Vitamin B12 and Folid acid deficiency present similarly. What is their difference and why?
**Neuro dysfunction** is only seen in VitB12 deficiency;
B12 deficiency --\> axonal demyelination in [peripheral n. / spinal cord (Posterior vs. Lateral column) / cerebrum]
28
[**G6PD** -Glucose 6 phosphate Dehydrogenase] deficiency
Mode of Inheritance
X-Linked recessive
29
Hereditary Spherocytosis
Mode of Inheritance
Auto Dom
30
Pts with [G6PD deficiency] are at risk for _____ when taking _____ before going to Africa. Peripheral blood smear will show \_\_\_\_\_
**medication induced oxidative stress**; **Antimalarials; Heinz Bodies** (dark RBC inclusions that stain with supravital stain)
31
Laboratory manifestation of [G6PD deficiency] (4)
intravascular and extravascular hemolysis with
anemia
reticulocytosis
[UIB - Unconjugated Indirect Bilirubinemia]
[low haptoglobin from DEC in HgB-Haptoglobin complexes]
32
Dactylitis (painful swelling of \_\_\_\_\_\_) is a common presentation of _____ in \_\_\_\_\_\_
Dactylitits (painful swelling of **Hands and Feet**) is a common presentation of **Sickle Cell** in **Kids**
33
[Sickle Cell Anemia] Labs (3)
INC [**UIB** - **U**nconjugated **I**ndirect **B**ilirubin]
INC Lactate Dehydrogenase
DEC Haptoglobin
34
[Sickle Cell Anemia]
Mode of Inheritance
auto recessive
## Footnote
*Most common auto recessive DO in Blacks*
35
List and Describe the 3 main pathogenic features of [Sickle Cell Anemia]
1. **Hemolysis**: RBC Sickling--\>Deformed RBC destruction by macrophages & mechanical stress = intra/EXtravascular hemolysis--\> Jaundice/Sclera Icterus. *Haptoglobin binds to INC free HgB preventing Renal injury*
2. **Vasoocclusion**: Hypoxic tissue injury from occlussion--\>Pain Crisis, Dactylitis (infarct in joint bones), Priapism and Autosplenectomy
3. **Infection:** Autosplenectomy --\> inability to fight encapsulated bacteria --\> [Staph & Salmonella Osteomyelitis]
36
Name the [Vitamin K-dependent Coagulation factors] (4), where they're made and why they're VitK-dependent
Factors 2/7/9/10; made in Liver
*7 has shortest half life*
These are initially made in liver and then activated by [VitK-dependent carboxylation]
37
Failure of ProThrombin Time to correct with VitK supplementation indicates \_\_\_\_\_\_\_
[Factor 7 deficiency from **Liver Dz** (Cirrhosis)]
38
Dermatomyositis Clinical Presentation (4)
Autoimmune Dz --\>
- [Proximal m. weakness (resembles polymyositis)]
- [PeriOrbital Heliotrope Rash]
- Gottron's Papules - *in image*
- [Mononuclear Perimysial infiltrates on histo]
39
Which CA is Dermatomyositis associated with (4)
1. Ovarian
2. Colorectal
3. Lung
4. Non-Hodgkin Lymphoma
## Footnote
*Gottron's Papules in image*
40
Clinical Presentation for [Hairy Cell Leukemia] (3)
B-Cell neoplasm --\>
1. Bone Marrow Failure --\> [Splenomegaly from reticuloendothelial infiltration]
2. Unsuccessful Bone marrow aspiration (Dry Tap)
3. Lymphocytes with cytoplasmic projections-*image*
41
Demographic for [Hairy Cell Leukemia], and how is it diagnosed?
[Middle Aged men]
## Footnote
[**TRAP** - **T**artrate **R**esistant **A**cid **P**hosphatase] Flow cytometry
42
[Factor 5 Leiden] MOD (2)
1. Factor 5 is genetically altered to resist protelysis/inactivation by [Activated Protein C] --\> [INC DVT/PE and Pregnancy Lost]
2. Factor 5 Leiden can **not** support APC's anticoagulant activity --\> [INC DVT/PE and Pregnancy Lost]
43
What's the most common cause of [Inherited Thrombophilia]?
Factor 5 Leiden
44
Labs for CML - **C**hronic **M**ylogenous **L**eukemia (2)
* [Elevated Myelocyte WBC]
* [**DECREASED** (Leukocyte alkaline phosphatase)] - *differentiates CML from Leukemoid rxn*
45
CML genetic cause (2)
PDGF mutations caused by:
Philadelphia Chromosome (translocation between Chromo 9 & 22)
vs.
BCR-ABL1 fusion gene
46
BCL2 function
Inhibits Apoptosis --\> promotes cell survival --\> (Follicular) Lymphoma
47
BCL2 oncogene is associated with \_\_\_\_\_\_. What's the MOD
BCL2 oncogene is associated with **Follicular Lymphoma**
Translocation of [BCL2 oncogene] from Chromo 18--\>[Chromo 14 IgHeavy Chain] --\> BCL2 Overexpression --\> inhibits too much apoptosis
48
[**SPEP** - **S**erum **P**rotein **E**lectro**P**horesis] showing elevated [gamma-globulin] usually represents a ___ consisting of overproduced \_\_\_\_\_. What Dz is this?
**SPEP** showing elevated [gamma-globulin] usually represents a **M protein** consisting of overproduced [**monoclonal immunoglobulin**] = **MULTIPLE MYELOMA**
49
Multiple Myeloma Clinical Presentation (6)
**C**razy **A**ss **BUMP**
1. [**C**RAB organ damage]: hyper*C*alcemia/ (*R*enal insufficiency from Bence-Jones PrOteinuria) / *A*nemia / [*B*one Dark Lytic Lesions & *B*ack Pain]
2. [**A**L amyloidosis]-from monoclonal (Ig light chains)
3. **B**one marrow w/plasmacytosis - *shown in image*
4. **U**rine IgG
5. [**M**ott cells on Histo]
6. **P**roteins (M Protein) in serum
50
Triad Presentation for [**PNH** - Paroxysmal Nocturnal Hemoglobinuria]
**PNH**
1. [**H**emolytic Anemia --\> Hemoglobinuria & Potential Renal Hemosiderosis (iron deposit)]
2. [**P**ancytopenia from stem cell damage]
3. [**N**ovel (atypical) Thrombosis locations (mesenteric vein)]
51
[Paroxsymal Nocturnal Hemoglobinuria] Cause and how it affects complement
[Complement-mediated hemolysis] caused by a [mutated PIGA gene] which normally makes [GPI anchor protein].
[GPI anchor protein] anchors markers like [CD55 Decay accelerating factor] and [CD59 MAC inhibitory protein] which both inactivate complement
52
How do [ProInflammatory IL1 & IF-gamma] affect VEGF
Both INC VEGF expression --\> promotes angiogenesis
53
[**FGF2** - **F**ibroblast **G**rowth **F**actor] Function (4)
1. Angiogenesis
2. Embryonic Development
3. Hematopoiesis
4. Wound Repair (recruits macrophages/fibroblast/endothelial cells)
54
What are [Schistocyte Helmet cells] and what are they caused by (2)
Fragmented RBC caused by
1. [HUS HAT microangiopathic hemolytic anemia]
vs.
2. [Prosthetic Cardiac Valves] - mechanical damage
* Both --\> Intravascular Hemolytic Anemia*
55
[Intravascular Hemolytic Anemia] Labs (3)
* DEC Haptoglobin (due to Hptoglbn binding to INC free Hgb)
* INC LDH
* INC [Unconjugated indirect Bilirubin]
56
Aplastic Anemia MOD
[Hematopoietic Stem Cell Deficiency (CD34)] --\> Bone Marrow Failure
57
Aplastic Anemia Causes (4)
58
Aplastic Anemia Clinical Presentation (4)
1. **NO** Splenomegaly
2. Anemia
3. Thrombocytopenia
4. Leukopenia
59
How do you diagnose Aplastic Anemia
60
Polycythemia Vera MOD
Uncontrolled RBC production due to [JAK2 mutation]
61
Polycythemia Vera Clinical Presentation (6)
1. Aquagenic Pruritus
2. Facial Plethora
3. Splenomegaly
4. Gouty Arthritis
5. RBC Mass INC - *Lab*
6. [Thrombocytosis & leukocytosis] - *Lab*
62
[TTP-HUS] MOD
[DEC ADAM-TS13 protease] --\> [Uncleaved Large vWF multimers] --\> Diffuse [microvascular platelet **thrombi**] --\> [HUS HAT] and thrombocytopenia
63
[TTP-HUS] Clinical Presentation (4)
1. [Hemolytic Anemia with (Schistocyte Helmet Cells)]
2. Thrombocytopenia
3. Renal Failure
4. Neurologic Sx
64
Identify the Cell and what Dz it's associated with
[**RADC - R**eactive **A**typical **D**owney **C**D8] Cell -*Controls EBV infection*
65
[\_\_\_\_ Deficiency] commonly occurs with Alcoholism and --\> ____ anemia from \_\_\_\_\_
[**Folic Acid** Deficiency] commonly occurs with Alcoholism and --\> **Megaloblastic Anemia** from impaired DNA base synthesis
## Footnote
*VitB12 can also be deficient*
66
Folic Acid and [VitB12/Cobalamin] deficiencies are both associated with Alcohol. How can you differentiate?
Folic **A**cid Deficiency 2° to EtOH = **A**cute usage (onset within weeks)
[VitB12/**C**obalamin] Deficiency 2° to EtOH = **C**hronic usage
67
MOA for how [**R**etino**B**lastoma gene] contributes to CA
Proliferation signals activate CDK4--\> [Hyperphosphorylates Rb protein] --\> prevents Rb from binding to [E2F tx factor].
[E2F tx factor] promotes cells to go from G1--\> S --\> [Division by DNA polymerase]!
*[G0 resting cells] contain active hypOphosphorylated Rb*
68
Von Willebrand Dz
MOD
Qualitative defect in [**platelet binding & aggregation**] --\> [Lifelong INC Bleeding time from Skin & Mucosal(menorrhagia vs. epistaxis)]
## Footnote
*Pts have normal Platelet Count*
69
How does [Von Willebrand Factor] normally function (2)
Promotes **Platelet Adhesion** by binding [Gp1B Platelet Glycoproteins] to [exposed Subendothelial Collagen]
AND
Acts as protective carrier protein for Factor 8 (INC its half life)
70
Why is HgB A2 elevated in beta-thalassemia?
[Beta-globin chain] underproduction --\> DEC HgB A1 synthesis--\> elevated HgB A2 to compensate
## Footnote
*Remember that HgBA1C is used to measure DM glycemic control*
71
How does TNF-a (AKA \_\_\_\_) affect Metabolism (2). What cell secretes it?
1. Suppresses appetite
2. INC BMR
## Footnote
* TNF-A (AKA **Cachectin**) --\> paraneoplastic Cachexia*
* Secreted by macrophages*
72
Describe the following levels for [**Beta Thalassemia minor**]
A: HgB A**1**
B: HgB A**2**
C: HgB **Fetal**
D: HgB **S**
73
Describe the following levels for [**Beta Thalassemia MAJOR**]
A: HgB A**1**
B: HgB A**2**
C: HgB **Fetal**
D: HgB **S**
74
Describe the following levels for [Sickle Cell **t****rait**]
A: HgB A**1**
B: HgB A**2**
C: HgB **Fetal**
D: HgB **S**
75
Describe the following levels for [Sickle Cell **DZ**]
A: HgB A**1**
B: HgB A**2**
C: HgB **Fetal**
D: HgB **S**
76
[Von Willebrand Dz] Mode of Inheritance
AUTO DOM + variable penetrance
77
What's the most common inherited bleeding DO
Von Willebrand DZ
## Footnote
*AUTO DOM + variable penetrance*
78
Clinical Significance of [Microsomal Monooxygenase]
Apart of the Cytochrome P450 system, and converts Pro-Carcinogens --\> Carcinogens--\> DNA mutations--\> CA
79
Which immunophenotype does [Precursor **B** ALL - **A**cute **L**ymphocytic **L**eukemia] display (3)
TdT
CD10
CD19
*Image shows Bone Marrow Blast (Lymphoblast)*
80
Which immunophenotype does [Precursor **T** ALL - **A**cute **L**ymphocytic **L**eukemia] display (4)
TdT
CD1a
[CD2 thru 5] = Tcell markers
[CD7 & 8]
*Image shows Bone Marrow Blast (Lymphoblast)*
81
Identify the abnormality and describe its MOD
Saddle Pulmonary Embolus
Hypercoaguability --\> DVT--\>PE that straddles bifurcation of main pulmonary artery
82
[DIC **in pregnancy**] MOD
Placental Abruptio causes--\> release of [Tissue Factor Thromboplastin] into maternal circulation --\> rapid consumption of clotting factors/platelets (DIC) --\> Bleeding
83
In what conditions do you commonly see Target Cells? (4)
1. Thalassemia
2. [Iron Deficiency Anemia]
3. Obstructive Liver Dz
4. Asplenia
84
In what conditions do you commonly see Bite Cells and why?
Glucose 6 phosphate dehydrogenase deficiency (oxidant induced damage)
Splenic macrophages removes Heinz bodies from RBC --\> Bite Cells
85
In what conditions do you commonly see Teardrop Cells and why?
Myelofibrosis
Caused by RBC **squeezing thru fibrous strands** when bone marrow is replaced with fibrosis or CA
86
In what conditions do you commonly see Spherocytes? (3) Describe them
Hereditary Spherocytosis
[Autoimmune Hemolytic Anemia]
Burns
*Spherocytes are RBC without normal central pallor*
87
How does [Carbon Tetrachloride] affect the Liver (3)
**Free Radical Damage** after being oxidized by P450 system--\>
- Endoplasmic Reticulum swelling
- Mitochondria Destruction
- INC Cell membrane permeability
88
Clinical Manifestations of [Acute Intermittent Porphyria] (4)
1. [Reddish Port-Wine Urine]
2. Neuro Sx
3. Abd Pain
4. [Urine PBG & ALA]
89
[Acute Intermittent Porphyria] MOD
[**P**orpho**B**ilino**G**en deaminiase] deficiency
90
[Acute Intermittent Porphyria] Tx (2)
**IV** Glucose vs. Heme (inhibits ALA synthase)
91
[Acute **Pro**Myelocytic Leukemia] MOD
[Translocation from Chromo 15 --\>17] --\> [PML/RAR*alpha* fusion gene] which can't signal proper cell differentiation like normal [Retinoic Acid R]--\> [CA **and** DIC]
## Footnote
*Acute **Pro**Myelocytic Leukemia is the M3 variant of AML*
92
[Acute **Pro**Myelocytic Leukemia] tx
**ATRA**
(**A**ll **T**rans **R**etinoic **A**cid) - Vitamin A derivative
93
Which pathogen is associated with Burkitt Lymphoma
EBV (immunodeficient pts AND endemic)
94
MOD for how EBV causes _____ Lymphoma
EBV INC B-cell proliferation --\> INC risk of cMYC [Translocation from Chromo 8 --\> 14] --\> cMYC overexpression --\> **Burkitt** Lymphoma
95
Burkitt Lymphoma Histology (3)
1. HIGH Ki67 proliferation index
2. [Starry Sky appearance from benign macrophages]
3. Diffuse medium size lymphocytes w/basophilic cytoplasm
96
Polycythemia vera is a ____ dz of _____ cells. It is caused by a ______ mutation
Polycythemia Vera is a **Clonal Myeloproliferative** Dz of [**Pluripotent Hematopoietic stem cells**].
It is caused by a [**JAK2-V617-F mutation**] --\> makes hematopoietic stem cells more sensitive to growth factors
97
Polycythemia Vera Clinical Manifestation (3)
* INC RBC Mass
* INC plasma volume
* DEC EPO
98
Name the [Factor 10 inhibitors] (4)
Rivaro**X**aban
Api**X**aban
[Fondaparinu**X** - indirect & less effective]
[Heparin - indirect]
*These do NOT affect Thrombin Time*
99
Name the [Direct (Thrombin Factor 2A) inhibitors] (3)
**DAB** *Directly* on that *Thrombin*!
## Footnote
**D**abigatran
**A**rgatroban
**B**ivalirudin
100
What would you expect the [Mean Corpuscular HgB concentration] to be in [Hereditary Spherocytosis]?
INC since RBC will be mildly dehydrated
101
[Hereditary Spherocytosis] MOD
AUTO DOM loss of [Spectrin cytoskeleton] --\> Bleb formation --\> Destruction by Spleen and macrophages --\> [Less deformable Spheroidal RBC with dEC lifespan]
## Footnote
*These RBC will contain no central pallor or biconcavity*
102
MOD for [Beta-Thalassemia]. How is this related to ePO
Genetic DEC in beta-**globulin** translation --\> Excess alpha-globins--\> RBC membrane rigidity--\>[INC extravascular hemolysis by splenic macrophage] --\> [hypOchromic microcytic anemia].
EPO is released **in excess** to compensate --\> [Bone Marrow expansion] and [Extramedullary Hematopoiesis]
103
Hepcidin is made by the ____ to do what?
Which conditions stimulate Hepcidin release? (2)
Which DEC Hepcidin release? (2)
Liver; **Lower** iron levels by [blocking intestinal iron absorption] and [blocking macrophage iron release]
## Footnote
INC with High iron vs. inflammation
DEC with hypOxia or [INC erythropoiesis]
104
How is Citrate releated to Blood transfusions
Pts receiving GOE [5-6 L of whole blood] --\> High Serum Citrate --\> [Chelation/DEC of Ca+ and Mg+] --\> Paresthesias
105
How might a newborn suffering from Vitamin K deficiency present? (3)
*Impaired clotting factor carboxylation*
1. Intracranial hemorrhage
2. Bulging ANT Fontanelle
3. [Eyes driven downward & unable to track up]
106
Sickle Cell pts are at risk of developing ___ deficiency ---\> ____ due to INC RBC turnover
Sickle cell pts are at risk of developing **Folic Acid** deficiency --\> **Macrocytosis** due to INC RBC turnover
107
tPA is a ___ that can cause ____ in the heart from \_\_\_\_. How does tPA work?
tPA is a **Fibrinolytic** that can cause [**BENIGN Reperfusion Arrhythmia**] in the heart from **arterial re-opening**
*refer to image*
108
2 most common cases of Aplastic Anemia
1. Toxic/pathogenic
2. Autoimmune--\> Apoptosis of pluripotent stem cells
109
Aplastic Anemia Histology (2)
Bone marrow with **hypOcellularity** and **INC Adiposity**
*image with Normal compared to Aplastic Anemia*
110
Clinical Presentation of Fanconi Anemia (3)
1. Short stature
2. Absent Thumbs
3. CA INC Risk [Myelodysplastic syndrome and AML]
111
What is Fanconi Anemia
Inherited cause of Aplastic Anemia
*Image showing normal bone marrow compared to Aplastic Anemia*
112
[**MDS** - **M**yelo**D**ysplastic **S**yndrome] MOD
[Stem cell maturation defect] --\> ineffective differentiation --\> Pancytopenia
113
[**MDS** - **M**yelo**D**ysplastic **S**yndrome] Histology (2)
Bone marrow biopsy:
1. Hypercellularity
2. [Abnormally differentiated cells with **misshapen nuclei**]
114
[**MDS** - **M**yelo**D**ysplastic **S**yndrome] Demographic
GOE 65 yo
115
Describe Pancytopenia (3)
*Pan* cytopenia : **TAN**
**T**hrombocytopenia (petechiae, hemorrhage)
**A**nemia (fatigue, pallor)
**N**eutropenia (opportunistic infection)
116
Most common cause of [1° Hemochromatosis]
[HFE Protein mutations]
117
[HFE protein mutation] MOD
[HFE protein mutation] --\> [DEC Hepcidin from liver] AND [INC RBC DMT1 expression] --\> **iron overload**
118
Pts with [HFE protein mutation] are at risk for developing which 2 things?
1. Cirrhosis
2. Hepatocellular Carcinoma
119
What is Haptoglobin
Binds to [INC free HgB] preventing Renal injury
120
What would the histo of Megaloblastic Anemia show? (3)
1. [Neutrophils with hypersegmented nuclei]
2. Macrocytosis
3. Ovalocytosis
121
What's the most important Pgn factor for [Bladder Urothelilal Transitional cell carcinoma]
Tumor Penetration into Bladder Wall
122
[Precursor **T** ALL] Clinical Manifestation (3)
Mediastinal mass --\>
- Respiratory sx (stridor/dyspnea)
- Dysphagia
- [Superior Vena Cava Syndrome]
* Image shows Bone Marrow Blast (Lymphoblast)*
123
[Precursor **T** ALL] Demographic
Males 8 - 20 yo
*Image shows Bone Marrow Blast (Lymphoblast)*
124
Identify the Poison based on Peripheral Blood Smear (2)
**Lead vs. Arsenic Poisoning**
*Basophilic Stippling + [Microcytic hypOchromic anemia]*
125
Dx for Lead Poisoning (2)
[Basophilic Stippling on Histo]
+
[Microcytic hypOchromic anemia]
126
List the Demographics associated with Lead Poisoning, and the pathognomonic physical exam finding (2)
1. [Children eating lead paint]
2. [Miners near Battery manufacturing]
PE: [(Blue Lead Lines) @ junction of teeth & gingivae]
127
Why is Pleomorphism, INC mitosis and nuclear changes not necessarily malignant **when in lymph nodes**
These are commonly seen in lymphocytes of [reactive hyperplastic lymph nodes]
## Footnote
*_Monoclonal_ gene rearrangements is a better indicator*
128
Identify and Describe
[Reed Sternberg Owl Eye cells] = large binucleated cells in the background of lymphocytic infiltrates
## Footnote
*Indicates Hodgkins Lymphoma*
129
Dz
**Hodgkins Lymphoma**
## Footnote
*[Reed Sternberg Owl Eye cells] = large binucleated cells in the background of lymphocytic infiltrates*
130
Vincristine SE (2)
1. [Neuropathy DL]
2. Vesicant
131
Vincristine Indications (2)
"Cristy helps *ALL* the *Non* Kids"
[Non-Hodgkin's Lymphoma]
ALL- Acute Lymphoblastic Leukemia
132
Vincristine MOA
Prevents Tubulin Polymerization
133
Chemotherapeutic (and toxic) effect of MTX can be overcome with what drug? Why does this help?
[Leucovorin THFolinic acid]; This is a reduced form of Folic Acid BUT doesn't require DHF reductase like Folic Acid
134
Which Drugs/Toxins cause Aplastic Anemia? (5)
135
How does Desmopressin alleviate bleeding?
Upregulates Factor 8 --\> Endothelial release of vWF(stabilized by Factor 8)
136
MTX can be used to treat ____ pregnancy. What's it's MOA
**Ectopic** Pregnancy;
Reversibly binds and inhibits [Dihydrofolate Reductase]--\> intermediate DHF accumulation
137
MTX Indications (7)
1. Brain Tumor - HIGH IV DOSES
2. Meningitis (carcinomatous vs. lymphomatous)
3. Leukemia
4. Lymphoma
5. Psoriasis
6. RA
7. Ectopic Pregnancy
138
Rasburicase MOA
Urate Oxidase that converts Uric acid --\> [Urine soluble **Allantoin**]
139
Rasburicase Indication
Treats [Hyperuricemia 2° to Tumor Lysis Syndrome]
140
[Tumor Lysis Syndrome] MOA and Clinical Presentation (4)
Develops during Chemo in CA with rapid turnover
HYPER**kup** + hypOcalcemia
1. HYPER**p**hosphatemia
2. HYPER**k**alemia
3. HYPER**u**ricemia (tx: Rasburicase vs. Allopurinol)
4. hypOcalcemia
141
Heparin MOA
Potentiates **naturally occuring AT3** to inhibit [Factor 10A and (Thrombin Factor 2A)]
142
[**LMWH**eparin] MOA
Potentiates **naturally occuring AT3** to inhibit [Factor 10A]
143
Heparin MOA
DVT Px (espeically hip & Knee surgery)
144
The MDR1 gene codes for the \_\_\_\_\_, which is a \_\_\_\_\_
MDR1 gene codes for [**P-GlycoProtein Efflux Pump**], which is an transmembrane ATP-dependent protein that effluxes hydrophobic protein out of the cell --\> ChemoResistance
145
Name the [**LMWH**eparin] drug
Enoxaparin
146
Hemorrhagic Cystitis is caused by which Chemo drugs? (2) What's additionally given to prevent this? (3)
Cyclophosphamide & Ifosfamide
Px =1. [Mesna(binds urine acrolein)]
2. Hydration
3. Bladder irrigation
147
Hydroxyurea Indication (3)
* **Sickle Cell Anemia** (INC HbB Fetal synthesis)
* Rapidly DECREASES High [WBC Blast count] in pts with **AML** and [**Chronic Granulocytic Leukemia w/blast crisis**]
*Inhibits [Ribonucleotide Reductase] --\> inhibits [DNA thymine] synthesis*
148
Hydroxyurea MOA
Inhibits [Ribonucleotide Reductase] --\> inhibits [DNA thymine] synthesis
*MOA for Sickle Cell Anemia Unknown*
149
HER2Neu is a ____ receptor overexpressed in ____ CA. _____ monoclonal Ab targets this receptor
[HER2Neu = **H**uman **E**pidermal growth factor **R**eceptor] is a **Tyrosine Kinase** receptor overxpressed in **Breast** CA.
**Trastuzumab** monoclonal Ab targets this receptor--\> promotes apoptosis
150
Which CA drugs require activation by HGPRT (2)
6-MP
&
6-thioguanine
151
6-MercaptoPurine Indication
A L L
152
6-MercaptoPurine Indication
6-MP is Degraded by [Hepatic Xanthine Oxidase] (*and XO can be degraded by Allopurinol)*
153
Which SE, regardless of tolerance, are _persistently_ present with Chronic Opioid use (2)
**Constipation** (Px: Laxatives & Hydration)
Miosis
154
Rivaro**X**aban Indication (2)
1. DVT
2. AFib
155
Rituximab MOA
Ab against **CD20** Lymphomas
156
Examples of [Anticholinergic antiEmetics]
157
Indication for [Anticholinergic antiEmetics]
158
Examples of [Antihistamine antiEmetics] (3)
159
Indication for [Antihistamine antiEmetics]
160
Examples of [(Dopamine R Blocker) antiEmetics] (2)
161
Indication for [(Dopamine R Blocker) antiEmetics]
162
Examples of [(Serotonin 5HT3 R Blocker) antiEmetics] (2)
163
Indication for [(Serotonin 5HT3 R Blocker) antiEmetics]
164
Examples of [(Neurokinin 1 R Blocker) antiEmetics] (2)
165
Indication for [(Neurokinin 1 Blocker) antiEmetics]
166
Multiple Myeloma [Tx and its MOA]
**C**razy **A**ss **BUMP**
[Bortezomib Proteasome inhibitor] = Boronic acid-containing protein that inhibts Proteasome --\> DEC protein production from neoplastic plasma cells--\> Apoptosis
167
Warfarin OD tx (2)
168
Heparin OD tx
169
Why is [**F**resh **F**rozen **P**lasma] contraindicated in Heparin OD
170
[Aspirin ASA] MOA
Irreversible COX1 and COX2 inhibitor --\> DEC Prostaglandin H2
171
Which Cell Cycle is Vincristine active in?
*Prevents Tubulin Polymerization*
172
Which Cell Cycle is 6-MercaptoPurine active in?
173
Glanzmann Thrombasthenia MOD
Deficient [GP 2B 3A] receptor which normally allows platelets to bind to fibrinogen and aggregate
174
Name the [GP 2B 3A] receptor Blockers (3)
1. Abciximab
2. Eptifibatide
3. Tirofiban
175
[Bernard Soulier Syndrome] MOD
GP1B DEC --\> Defect in Platelet binding to vWF --\> Defective **Plug formation**
176
Name the drugs that are [Platelet ADP R Blockers] (3)
**C**an't **P**lace (GP 2B 3A) on **T**op
**C**lopidogrel
**P**rasugrel
**T**iclopidine
177
Etoposide MOA
E*2*oposide: Topoisomerase *2* inhibitor --\> Double stranded DNA breaks
178
Etoposide Indication (3)
1. Testicular CA
2. Lymphoma
3. SOLC
179
Etoposide SE
Leukomogenic (Leukemia Promoter)
180
Ganciclovir has a severe SE of ____ that is exacerbated with what 2 drugs?
Ganciclovir has a severe SE of **Neutrophenia** ; worsened with
**Zidovudine**
or
**Bactrim**
181
Celecoxib MOA
Selective COX2 only inhibitor --\> DEC inflammation/pain
## Footnote
*platelet aggregation and GI protection remain intact*
182
Function of Ristocetin
Activates [GP1B Platelet R] to **make them** **available** for vWF to adhere them to exposed subendothelial collagen
183
Sickle Cell Anemia Clinical Presentation (3)
1. Exeritional Dyspnea
2. PNA --\> Acute Chest Syndrome
3. Bone Pain
184
Sickle Cell Anemia Genetic MOD
point mutation --\> Valine being substituted for Glutamic acid in the 6th position of the [HgB **B** chain]
185
Which enzyme deficiency of Heme Synthesis actually involves Photosensitivity and what is this called? and how will this manifest?
[**UROD** - UROPORPHYRINOGEN DECARBOXYLASE deaminiase] deficiency --\> [Porphyria Cutanea Tarda]
Photosensitivity --\> [Vesicle & Blister formation] in sun-expoed areas
186
How does [Porphyria Cutanea Tarda] manifest?
**Photosensitivity** --\> [Vesicle & Blister formation] in sun-expoed areas (*not seen in Acute intermittent porphyria*)
## Footnote
[Porphyria Cutanea Tarda] = Uroporphyrinogen Decarboxylase Deficiency or UROD
187
[**EBRT** - **E**xternal **B**eam **R**adiation **T**herapy] MOD (2)
ionizing radiation causes Double stranded DNA breaks and formation of O2 Free Radicals
188
How is [Pyruvate Kinase Deficiency] related to Red Pulp Hyperplasia
PK Deficiency --\> Failure of Glycolysis--\>No ATP to maintain RBC structure--\> OVERWORK from spleen to remove the deformed RBC --\> Splenomegaly = Red Pulp Hyperplasia
189
What is the surface marker for Macrophages
CD14
190
How does [inhaled amyl nitrite] treate Cyanide Poisoning
Oxidizes HgB [Ferrous 2+]--\> [Ferric 3+] = Methemeglobin. Methemoglobin can't carry O2 but _can_ pull cyanide off of [mitochondrial cytochrome oxidase] and limit its effects
191
Cyanide Poisoning Tx (3)
1. [Inhaled Amyl Nitrite]
2. HydroxyCoBalamin (VitB12 precursor) - *excretes Cyanide in urine*
3. [Na+ Thiosulfate] -*excretes Cyanide in urine*
192
Describe the Process of Heme --\> Brown Feces (6)
