Heme - Onc Flashcards

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1
Q

Extramedullary Hematopoiesis is most commonly caused by what?

A

[Beta-thalassemia] = [Chronic Hemolytic Anemia]

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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)

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3
Q

Name 4 Common causes of Vitamin B12 Deficiency

A
  1. GIP [Gastritis / Ilial Resection / Pancreatitis]
  2. Fish Tapeworm
  3. Atrophic Gastritis –> Destruction of [Parietal Cells that produce IF] –> DEC [Intrinsic Factor]
  4. Strict Vegetarians (Proteins contain Vit B12)
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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

  1. Butterfly Malor Rash
  2. Ulcers (Oral vs. Nasal)
  3. Brain Dz (seizures / psychosis)
  4. Arthritis (can tx w/anti-malarial)
  5. Serositis (pericarditis / pleurisy / peritonitis)
  6. Kidney Dz (Lupus Membranous Nephropathy) vs. (Lupus Nephritis)
  7. Anti-nuclear Antibodies
  8. Pancytopenia (includes compliment)
  9. Photosensitivity
  10. Autoantibodies (dsDNA vs. Smith vs. Phospholipid(False positive VLDR) )
  11. Discoid Rash anywhere on body

B: At least 4

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5
Q

Where does Extramedullary Hematopoiesis for this condition occur? (2)

A

Liver and Spleen

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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

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7
Q

Key Features of [Ductal Carcinoma In Situ] (3)

Invasive or NonInvasive?

A
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8
Q

Key Features of [Paget Breast CA] (2)

Invasive or NonInvasive?

A
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9
Q

Key Features of [Ductal Carcinoma] (2)

Invasive or NonInvasive?

A
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10
Q

Key Features of [Lobular Carcinoma] (2)

Invasive or NonInvasive?

A
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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.

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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

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13
Q

Manifestations of Atrophic Gastritis (3)

A
  • hypOchlorhydria
  • DEC IF production –> VitB12 Deficiency
  • INC methylmalonic acid
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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]

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15
Q

Von Willebrand Dz

A: Platelet Count

B: PT time

C: aPTT time

D: Fibrinogen level

E: Describe Peripheral Blood Smear

A
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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
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17
Q

TTP-HUS

A: Platelet Count

B: PT time

C: aPTT time

D: Fibrinogen level

E: Describe Peripheral Blood Smear

A
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18
Q

DIC

A: Platelet Count

B: PT time

C: aPTT time

D: Fibrinogen level

E: Describe Peripheral Blood Smear

A
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19
Q

[IITP - Immune Idiopathic Thrombocytopenic Purpura]

MOD

A

Autoantibodies destroy platelets –> Isolated Thrombocytopenia w/ NO splenomegaly

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20
Q

Clinical Sx for [Systemic Lupus Erythematosus] (8)

A
  1. Constitutional (Fever / Fatigue / Wt. Loss)
  2. Arthritis -Symmetric & Migratory
  3. [Malor Butterfly Rash]
  4. Photosensitivity
  5. Serositits (pericarditis & peritonitits)
  6. Thromboembolism (due to antiphospholipid Ab)
  7. Seizures & Cognition DEC
  8. [Lupus Nephritis = PrOteinuria + INC Creatinine (Type 3) Hypersensitivity]
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21
Q

Labs for [Systemic Lupus Erythematosus] (4)

A
  1. Pancytopenia (Hemolytic Anemia Type 2 Hypersensitivity) / Thrombocytopenia / Leukopenia)
  2. hypOcomplent (C3 & C4)
  3. [ANA Ab (sensitive)]
  4. [Anti-dsDNA and SM (specific)]
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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

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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

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24
Q

Name the CA that cause [OsteoBLastic Bone Metastases] (3)

A

Prostate

SOLC

Hodgkin Lymphoma

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25
Q

Name the CA that cause BOTH [OsteoBLastic and Osteolytic Bone Metastases] (2)

A

GI

Breast

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26
Q

Name the CA that cause [Osteolytic Bone Metastases] (5)

A
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27
Q

Vitamin B12 and Folid acid deficiency present similarly. What is their difference and why?

A

Neuro dysfunction is only seen in VitB12 deficiency;

B12 deficiency –> axonal demyelination in [peripheral n. / spinal cord (Posterior vs. Lateral column) / cerebrum]

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28
Q

[G6PD -Glucose 6 phosphate Dehydrogenase] deficiency

Mode of Inheritance

A

X-Linked recessive

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29
Q

Hereditary Spherocytosis

Mode of Inheritance

A

Auto Dom

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30
Q

Pts with [G6PD deficiency] are at risk for _____ when taking _____ before going to Africa. Peripheral blood smear will show _____

A

medication induced oxidative stress; Antimalarials; Heinz Bodies (dark RBC inclusions that stain with supravital stain)

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31
Q

Laboratory manifestation of [G6PD deficiency] (4)

A

intravascular and extravascular hemolysis with

anemia

reticulocytosis

[UIB - Unconjugated Indirect Bilirubinemia]

[low haptoglobin from DEC in HgB-Haptoglobin complexes]

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32
Q

Dactylitis (painful swelling of ______) is a common presentation of _____ in ______

A

Dactylitits (painful swelling of Hands and Feet) is a common presentation of Sickle Cell in Kids

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33
Q

[Sickle Cell Anemia] Labs (3)

A

INC [UIB - Unconjugated Indirect Bilirubin]

INC Lactate Dehydrogenase

DEC Haptoglobin

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34
Q

[Sickle Cell Anemia]

Mode of Inheritance

A

auto recessive

Most common auto recessive DO in Blacks

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35
Q

List and Describe the 3 main pathogenic features of [Sickle Cell Anemia]

A
  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]
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36
Q

Name the [Vitamin K-dependent Coagulation factors] (4), where they’re made and why they’re VitK-dependent

A

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]

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37
Q

Failure of ProThrombin Time to correct with VitK supplementation indicates _______

A

[Factor 7 deficiency from Liver Dz (Cirrhosis)]

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38
Q

Dermatomyositis Clinical Presentation (4)

A

Autoimmune Dz –>

  • [Proximal m. weakness (resembles polymyositis)]
  • [PeriOrbital Heliotrope Rash]
  • Gottron’s Papules - in image
  • [Mononuclear Perimysial infiltrates on histo]
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39
Q

Which CA is Dermatomyositis associated with (4)

A
  1. Ovarian
  2. Colorectal
  3. Lung
  4. Non-Hodgkin Lymphoma

Gottron’s Papules in image

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40
Q

Clinical Presentation for [Hairy Cell Leukemia] (3)

A

B-Cell neoplasm –>

  1. Bone Marrow Failure –> [Splenomegaly from reticuloendothelial infiltration]
  2. Unsuccessful Bone marrow aspiration (Dry Tap)
  3. Lymphocytes with cytoplasmic projections-image
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41
Q

Demographic for [Hairy Cell Leukemia], and how is it diagnosed?

A

[Middle Aged men]

[TRAP - Tartrate Resistant Acid Phosphatase] Flow cytometry

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42
Q

[Factor 5 Leiden] MOD (2)

A
  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]
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43
Q

What’s the most common cause of [Inherited Thrombophilia]?

A

Factor 5 Leiden

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44
Q

Labs for CML - Chronic Mylogenous Leukemia (2)

A
  • [Elevated Myelocyte WBC]
  • [DECREASED (Leukocyte alkaline phosphatase)] - differentiates CML from Leukemoid rxn
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45
Q

CML genetic cause (2)

A

PDGF mutations caused by:

Philadelphia Chromosome (translocation between Chromo 9 & 22)

vs.

BCR-ABL1 fusion gene

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46
Q

BCL2 function

A

Inhibits Apoptosis –> promotes cell survival –> (Follicular) Lymphoma

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47
Q

BCL2 oncogene is associated with ______. What’s the MOD

A

BCL2 oncogene is associated with Follicular Lymphoma

Translocation of [BCL2 oncogene] from Chromo 18–>[Chromo 14 IgHeavy Chain] –> BCL2 Overexpression –> inhibits too much apoptosis

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48
Q

[SPEP - Serum Protein ElectroPhoresis] showing elevated [gamma-globulin] usually represents a ___ consisting of overproduced _____. What Dz is this?

A

SPEP showing elevated [gamma-globulin] usually represents a M protein consisting of overproduced [monoclonal immunoglobulin] = MULTIPLE MYELOMA

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49
Q

Multiple Myeloma Clinical Presentation (6)

A

Crazy Ass BUMP

  1. [AL amyloidosis]-from monoclonal (Ig light chains)
  2. Bone marrow w/plasmacytosis - shown in image
  3. Urine IgG
  4. [Mott cells on Histo]
  5. Proteins (M Protein) in serum
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50
Q

Triad Presentation for [PNH - Paroxysmal Nocturnal Hemoglobinuria]

A

PNH

  1. [Hemolytic Anemia –> Hemoglobinuria & Potential Renal Hemosiderosis (iron deposit)]
  2. [Pancytopenia from stem cell damage]
  3. [Novel (atypical) Thrombosis locations (mesenteric vein)]
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51
Q

[Paroxsymal Nocturnal Hemoglobinuria] Cause and how it affects complement

A

[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

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52
Q

How do [ProInflammatory IL1 & IF-gamma] affect VEGF

A

Both INC VEGF expression –> promotes angiogenesis

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53
Q

[FGF2 - Fibroblast Growth Factor] Function (4)

A
  1. Angiogenesis
  2. Embryonic Development
  3. Hematopoiesis
  4. Wound Repair (recruits macrophages/fibroblast/endothelial cells)
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54
Q

What are [Schistocyte Helmet cells] and what are they caused by (2)

A

Fragmented RBC caused by

  1. [HUS HAT microangiopathic hemolytic anemia]

vs.

  1. [Prosthetic Cardiac Valves] - mechanical damage
    * Both –> Intravascular Hemolytic Anemia*
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55
Q

[Intravascular Hemolytic Anemia] Labs (3)

A
  • DEC Haptoglobin (due to Hptoglbn binding to INC free Hgb)
  • INC LDH
  • INC [Unconjugated indirect Bilirubin]
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56
Q

Aplastic Anemia MOD

A

[Hematopoietic Stem Cell Deficiency (CD34)] –> Bone Marrow Failure

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57
Q

Aplastic Anemia Causes (4)

A
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58
Q

Aplastic Anemia Clinical Presentation (4)

A
  1. NO Splenomegaly
  2. Anemia
  3. Thrombocytopenia
  4. Leukopenia
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59
Q

How do you diagnose Aplastic Anemia

A
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60
Q

Polycythemia Vera MOD

A

Uncontrolled RBC production due to [JAK2 mutation]

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61
Q

Polycythemia Vera Clinical Presentation (6)

A
  1. Aquagenic Pruritus
  2. Facial Plethora
  3. Splenomegaly
  4. Gouty Arthritis
  5. RBC Mass INC - Lab
  6. [Thrombocytosis & leukocytosis] - Lab
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62
Q

[TTP-HUS] MOD

A

[DEC ADAM-TS13 protease] –> [Uncleaved Large vWF multimers] –> Diffuse [microvascular platelet thrombi] –> [HUS HAT] and thrombocytopenia

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63
Q

[TTP-HUS] Clinical Presentation (4)

A
  1. [Hemolytic Anemia with (Schistocyte Helmet Cells)]
  2. Thrombocytopenia
  3. Renal Failure
  4. Neurologic Sx
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64
Q

Identify the Cell and what Dz it’s associated with

A

[RADC - Reactive Atypical Downey CD8] Cell -Controls EBV infection

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65
Q

[____ Deficiency] commonly occurs with Alcoholism and –> ____ anemia from _____

A

[Folic Acid Deficiency] commonly occurs with Alcoholism and –> Megaloblastic Anemia from impaired DNA base synthesis

VitB12 can also be deficient

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66
Q

Folic Acid and [VitB12/Cobalamin] deficiencies are both associated with Alcohol. How can you differentiate?

A

Folic Acid Deficiency 2° to EtOH = Acute usage (onset within weeks)

[VitB12/Cobalamin] Deficiency 2° to EtOH = Chronic usage

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67
Q

MOA for how [RetinoBlastoma gene] contributes to CA

A

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

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68
Q

Von Willebrand Dz

MOD

A

Qualitative defect in [platelet binding & aggregation] –> [Lifelong INC Bleeding time from Skin & Mucosal(menorrhagia vs. epistaxis)]

Pts have normal Platelet Count

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69
Q

How does [Von Willebrand Factor] normally function (2)

A

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)

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70
Q

Why is HgB A<strong>2</strong> elevated in beta-thalassemia?

A

[Beta-globin chain] underproduction –> DEC HgB A<strong>1</strong> synthesis–> elevated HgB A<strong>2</strong> to compensate

Remember that HgBA1C is used to measure DM glycemic control

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71
Q

How does TNF-a (AKA ____) affect Metabolism (2). What cell secretes it?

A
  1. Suppresses appetite
  2. INC BMR

  • TNF-A (AKA Cachectin) –> paraneoplastic Cachexia*
  • Secreted by macrophages*
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72
Q

Describe the following levels for [Beta Thalassemia minor]

A: HgB A1

B: HgB A2

C: HgB Fetal

D: HgB S

A
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73
Q

Describe the following levels for [Beta Thalassemia MAJOR]

A: HgB A1

B: HgB A2

C: HgB Fetal

D: HgB S

A
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74
Q

Describe the following levels for [Sickle Cell trait]

A: HgB A1

B: HgB A2

C: HgB Fetal

D: HgB S

A
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75
Q

Describe the following levels for [Sickle Cell DZ]

A: HgB A1

B: HgB A2

C: HgB Fetal

D: HgB S

A
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76
Q

[Von Willebrand Dz] Mode of Inheritance

A

AUTO DOM + variable penetrance

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77
Q

What’s the most common inherited bleeding DO

A

Von Willebrand DZ

AUTO DOM + variable penetrance

78
Q

Clinical Significance of [Microsomal Monooxygenase]

A

Apart of the Cytochrome P450 system, and converts Pro-Carcinogens –> Carcinogens–> DNA mutations–> CA

79
Q

Which immunophenotype does [Precursor B ALL - Acute Lymphocytic Leukemia] display (3)

A

TdT

CD10

CD19

Image shows Bone Marrow Blast (Lymphoblast)

80
Q

Which immunophenotype does [Precursor T ALL - Acute Lymphocytic Leukemia] display (4)

A

TdT

CD1a

[CD2 thru 5] = Tcell markers

[CD7 & 8]

Image shows Bone Marrow Blast (Lymphoblast)

81
Q

Identify the abnormality and describe its MOD

A

Saddle Pulmonary Embolus

Hypercoaguability –> DVT–>PE that straddles bifurcation of main pulmonary artery

82
Q

[DIC in pregnancy] MOD

A

Placental Abruptio causes–> release of [Tissue Factor Thromboplastin] into maternal circulation –> rapid consumption of clotting factors/platelets (DIC) –> Bleeding

83
Q

In what conditions do you commonly see Target Cells? (4)

A
  1. Thalassemia
  2. [Iron Deficiency Anemia]
  3. Obstructive Liver Dz
  4. Asplenia
84
Q

In what conditions do you commonly see Bite Cells and why?

A

Glucose 6 phosphate dehydrogenase deficiency (oxidant induced damage)

Splenic macrophages removes Heinz bodies from RBC –> Bite Cells

85
Q

In what conditions do you commonly see Teardrop Cells and why?

A

Myelofibrosis

Caused by RBC squeezing thru fibrous strands when bone marrow is replaced with fibrosis or CA

86
Q

In what conditions do you commonly see Spherocytes? (3) Describe them

A

Hereditary Spherocytosis

[Autoimmune Hemolytic Anemia]

Burns

Spherocytes are RBC without normal central pallor

87
Q

How does [Carbon Tetrachloride] affect the Liver (3)

A

Free Radical Damage after being oxidized by P450 system–>

  • Endoplasmic Reticulum swelling
  • Mitochondria Destruction
  • INC Cell membrane permeability
88
Q

Clinical Manifestations of [Acute Intermittent Porphyria] (4)

A
  1. [Reddish Port-Wine Urine]
  2. Neuro Sx
  3. Abd Pain
  4. [Urine PBG & ALA]
89
Q

[Acute Intermittent Porphyria] MOD

A

[PorphoBilinoGen deaminiase] deficiency

90
Q

[Acute Intermittent Porphyria] Tx (2)

A

IV Glucose vs. Heme (inhibits ALA synthase)

91
Q

[Acute ProMyelocytic Leukemia] MOD

A

[Translocation from Chromo 15 –>17] –> [PML/RARalpha fusion gene] which can’t signal proper cell differentiation like normal [Retinoic Acid R]–> [CA and DIC]

Acute ProMyelocytic Leukemia is the M3 variant of AML

92
Q

[Acute ProMyelocytic Leukemia] tx

A

ATRA

(All Trans Retinoic Acid) - Vitamin A derivative

93
Q

Which pathogen is associated with Burkitt Lymphoma

A

EBV (immunodeficient pts AND endemic)

94
Q

MOD for how EBV causes _____ Lymphoma

A

EBV INC B-cell proliferation –> INC risk of cMYC [Translocation from Chromo 8 –> 14] –> cMYC overexpression –> Burkitt Lymphoma

95
Q

Burkitt Lymphoma Histology (3)

A
  1. HIGH Ki67 proliferation index
  2. [Starry Sky appearance from benign macrophages]
  3. Diffuse medium size lymphocytes w/basophilic cytoplasm
96
Q

Polycythemia vera is a ____ dz of _____ cells. It is caused by a ______ mutation

A

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
Q

Polycythemia Vera Clinical Manifestation (3)

A
  • INC RBC Mass
  • INC plasma volume
  • DEC EPO
98
Q

Name the [Factor 10 inhibitors] (4)

A

RivaroXaban

ApiXaban

[FondaparinuX - indirect & less effective]

[Heparin - indirect]

These do NOT affect Thrombin Time

99
Q

Name the [Direct (Thrombin Factor 2A) inhibitors] (3)

A

DAB Directly on that Thrombin!

Dabigatran

Argatroban

Bivalirudin

100
Q

What would you expect the [Mean Corpuscular HgB concentration] to be in [Hereditary Spherocytosis]?

A

INC since RBC will be mildly dehydrated

101
Q

[Hereditary Spherocytosis] MOD

A

AUTO DOM loss of [Spectrin cytoskeleton] –> Bleb formation –> Destruction by Spleen and macrophages –> [Less deformable Spheroidal RBC with dEC lifespan]

These RBC will contain no central pallor or biconcavity

102
Q

MOD for [Beta-Thalassemia]. How is this related to ePO

A

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
Q

Hepcidin is made by the ____ to do what?

Which conditions stimulate Hepcidin release? (2)

Which DEC Hepcidin release? (2)

A

Liver; Lower iron levels by [blocking intestinal iron absorption] and [blocking macrophage iron release]

INC with High iron vs. inflammation

DEC with hypOxia or [INC erythropoiesis]

104
Q

How is Citrate releated to Blood transfusions

A

Pts receiving GOE [5-6 L of whole blood] –> High Serum Citrate –> [Chelation/DEC of Ca+ and Mg+] –> Paresthesias

105
Q

How might a newborn suffering from Vitamin K deficiency present? (3)

A

Impaired clotting factor carboxylation

  1. Intracranial hemorrhage
  2. Bulging ANT Fontanelle
  3. [Eyes driven downward & unable to track up]
106
Q

Sickle Cell pts are at risk of developing ___ deficiency —> ____ due to INC RBC turnover

A

Sickle cell pts are at risk of developing Folic Acid deficiency –> Macrocytosis due to INC RBC turnover

107
Q

tPA is a ___ that can cause ____ in the heart from ____. How does tPA work?

A

tPA is a Fibrinolytic that can cause [BENIGN Reperfusion Arrhythmia] in the heart from arterial re-opening

refer to image

108
Q

2 most common cases of Aplastic Anemia

A
  1. Toxic/pathogenic
  2. Autoimmune–> Apoptosis of pluripotent stem cells
109
Q

Aplastic Anemia Histology (2)

A

Bone marrow with hypOcellularity and INC Adiposity

image with Normal compared to Aplastic Anemia

110
Q

Clinical Presentation of Fanconi Anemia (3)

A
  1. Short stature
  2. Absent Thumbs
  3. CA INC Risk [Myelodysplastic syndrome and AML]
111
Q

What is Fanconi Anemia

A

Inherited cause of Aplastic Anemia

Image showing normal bone marrow compared to Aplastic Anemia

112
Q

[MDS - MyeloDysplastic Syndrome] MOD

A

[Stem cell maturation defect] –> ineffective differentiation –> Pancytopenia

113
Q

[MDS - MyeloDysplastic Syndrome] Histology (2)

A

Bone marrow biopsy:

  1. Hypercellularity
  2. [Abnormally differentiated cells with misshapen nuclei]
114
Q

[MDS - MyeloDysplastic Syndrome] Demographic

A

GOE 65 yo

115
Q

Describe Pancytopenia (3)

A

Pan cytopenia : TAN

Thrombocytopenia (petechiae, hemorrhage)

Anemia (fatigue, pallor)

Neutropenia (opportunistic infection)

116
Q

Most common cause of [1° Hemochromatosis]

A

[HFE Protein mutations]

117
Q

[HFE protein mutation] MOD

A

[HFE protein mutation] –> [DEC Hepcidin from liver] AND [INC RBC DMT1 expression] –> iron overload

118
Q

Pts with [HFE protein mutation] are at risk for developing which 2 things?

A
  1. Cirrhosis
  2. Hepatocellular Carcinoma
119
Q

What is Haptoglobin

A

Binds to [INC free HgB] preventing Renal injury

120
Q

What would the histo of Megaloblastic Anemia show? (3)

A
  1. [Neutrophils with hypersegmented nuclei]
  2. Macrocytosis
  3. Ovalocytosis
121
Q

What’s the most important Pgn factor for [Bladder Urothelilal Transitional cell carcinoma]

A

Tumor Penetration into Bladder Wall

122
Q

[Precursor T ALL] Clinical Manifestation (3)

A

Mediastinal mass –>

  • Respiratory sx (stridor/dyspnea)
  • Dysphagia
  • [Superior Vena Cava Syndrome]
  • Image shows Bone Marrow Blast (Lymphoblast)*
123
Q

[Precursor T ALL] Demographic

A

Males 8 - 20 yo

Image shows Bone Marrow Blast (Lymphoblast)

124
Q

Identify the Poison based on Peripheral Blood Smear (2)

A

Lead vs. Arsenic Poisoning

Basophilic Stippling + [Microcytic hypOchromic anemia]

125
Q

Dx for Lead Poisoning (2)

A

[Basophilic Stippling on Histo]

+

[Microcytic hypOchromic anemia]

126
Q

List the Demographics associated with Lead Poisoning, and the pathognomonic physical exam finding (2)

A
  1. [Children eating lead paint]
  2. [Miners near Battery manufacturing]

PE: [(Blue Lead Lines) @ junction of teeth & gingivae]

127
Q

Why is Pleomorphism, INC mitosis and nuclear changes not necessarily malignant when in lymph nodes

A

These are commonly seen in lymphocytes of [reactive hyperplastic lymph nodes]

Monoclonal gene rearrangements is a better indicator

128
Q

Identify and Describe

A

[Reed Sternberg Owl Eye cells] = large binucleated cells in the background of lymphocytic infiltrates

Indicates Hodgkins Lymphoma

129
Q

Dz

A

Hodgkins Lymphoma

[Reed Sternberg Owl Eye cells] = large binucleated cells in the background of lymphocytic infiltrates

130
Q

Vincristine SE (2)

A
  1. [Neuropathy DL]
  2. Vesicant
131
Q

Vincristine Indications (2)

A

“Cristy helps ALL the Non Kids”

[Non-Hodgkin’s Lymphoma]

ALL- Acute Lymphoblastic Leukemia

132
Q

Vincristine MOA

A

Prevents Tubulin Polymerization

133
Q

Chemotherapeutic (and toxic) effect of MTX can be overcome with what drug? Why does this help?

A

[Leucovorin THFolinic acid]; This is a reduced form of Folic Acid BUT doesn’t require DHF reductase like Folic Acid

134
Q

Which Drugs/Toxins cause Aplastic Anemia? (5)

A
135
Q

How does Desmopressin alleviate bleeding?

A

Upregulates Factor 8 –> Endothelial release of vWF(stabilized by Factor 8)

136
Q

MTX can be used to treat ____ pregnancy. What’s it’s MOA

A

Ectopic Pregnancy;

Reversibly binds and inhibits [Dihydrofolate Reductase]–> intermediate DHF accumulation

137
Q

MTX Indications (7)

A
  1. Brain Tumor - HIGH IV DOSES
  2. Meningitis (carcinomatous vs. lymphomatous)
  3. Leukemia
  4. Lymphoma
  5. Psoriasis
  6. RA
  7. Ectopic Pregnancy
138
Q

Rasburicase MOA

A

Urate Oxidase that converts Uric acid –> [Urine soluble Allantoin]

139
Q

Rasburicase Indication

A

Treats [Hyperuricemia 2° to Tumor Lysis Syndrome]

140
Q

[Tumor Lysis Syndrome] MOA and Clinical Presentation (4)

A

Develops during Chemo in CA with rapid turnover

HYPERkup + hypOcalcemia

  1. HYPERphosphatemia
  2. HYPERkalemia
  3. HYPERuricemia (tx: Rasburicase vs. Allopurinol)
  4. hypOcalcemia
141
Q

Heparin MOA

A

Potentiates naturally occuring AT3 to inhibit [Factor 10A and (Thrombin Factor 2A)]

142
Q

[LMWHeparin] MOA

A

Potentiates naturally occuring AT3 to inhibit [Factor 10A]

143
Q

Heparin MOA

A

DVT Px (espeically hip & Knee surgery)

144
Q

The MDR1 gene codes for the _____, which is a _____

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
Q

Name the [LMWHeparin] drug

A

Enoxaparin

146
Q

Hemorrhagic Cystitis is caused by which Chemo drugs? (2) What’s additionally given to prevent this? (3)

A

Cyclophosphamide & Ifosfamide

Px =1. [Mesna(binds urine acrolein)]

  1. Hydration
  2. Bladder irrigation
147
Q

Hydroxyurea Indication (3)

A
  • 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
Q

Hydroxyurea MOA

A

​Inhibits [Ribonucleotide Reductase] –> inhibits [DNA thymine] synthesis

MOA for Sickle Cell Anemia Unknown

149
Q

HER2Neu is a ____ receptor overexpressed in ____ CA. _____ monoclonal Ab targets this receptor

A

[HER2Neu = Human Epidermal growth factor Receptor] is a Tyrosine Kinase receptor overxpressed in Breast CA.

Trastuzumab monoclonal Ab targets this receptor–> promotes apoptosis

150
Q

Which CA drugs require activation by HGPRT (2)

A

6-MP

&

6-thioguanine

151
Q

6-MercaptoPurine Indication

A

A L L

152
Q

6-MercaptoPurine Indication

A

6-MP is Degraded by [Hepatic Xanthine Oxidase] (and XO can be degraded by Allopurinol)

153
Q

Which SE, regardless of tolerance, are persistently present with Chronic Opioid use (2)

A

Constipation (Px: Laxatives & Hydration)

Miosis

154
Q

RivaroXaban Indication (2)

A
  1. DVT
  2. AFib
155
Q

Rituximab MOA

A

Ab against CD20 Lymphomas

156
Q

Examples of [Anticholinergic antiEmetics]

A
157
Q

Indication for [Anticholinergic antiEmetics]

A
158
Q

Examples of [Antihistamine antiEmetics] (3)

A
159
Q

Indication for [Antihistamine antiEmetics]

A
160
Q

Examples of [(Dopamine R Blocker) antiEmetics] (2)

A
161
Q

Indication for [(Dopamine R Blocker) antiEmetics]

A
162
Q

Examples of [(Serotonin 5HT3 R Blocker) antiEmetics] (2)

A
163
Q

Indication for [(Serotonin 5HT3 R Blocker) antiEmetics]

A
164
Q

Examples of [(Neurokinin 1 R Blocker) antiEmetics] (2)

A
165
Q

Indication for [(Neurokinin 1 Blocker) antiEmetics]

A
166
Q

Multiple Myeloma [Tx and its MOA]

A

Crazy Ass BUMP

[Bortezomib Proteasome inhibitor] = Boronic acid-containing protein that inhibts Proteasome –> DEC protein production from neoplastic plasma cells–> Apoptosis

167
Q

Warfarin OD tx (2)

A
168
Q

Heparin OD tx

A
169
Q

Why is [Fresh Frozen Plasma] contraindicated in Heparin OD

A
170
Q

[Aspirin ASA] MOA

A

Irreversible COX1 and COX2 inhibitor –> DEC Prostaglandin H2

171
Q

Which Cell Cycle is Vincristine active in?

A

Prevents Tubulin Polymerization

172
Q

Which Cell Cycle is 6-MercaptoPurine active in?

A
173
Q

Glanzmann Thrombasthenia MOD

A

Deficient [GP 2B 3A] receptor which normally allows platelets to bind to fibrinogen and aggregate

174
Q

Name the [GP 2B 3A] receptor Blockers (3)

A
  1. Abciximab
  2. Eptifibatide
  3. Tirofiban
175
Q

[Bernard Soulier Syndrome] MOD

A

GP1B DEC –> Defect in Platelet binding to vWF –> Defective Plug formation

176
Q

Name the drugs that are [Platelet ADP R Blockers] (3)

A

Can’t Place (GP 2B 3A) on Top

Clopidogrel

Prasugrel

Ticlopidine

177
Q

Etoposide MOA

A

E2oposide: Topoisomerase 2 inhibitor –> Double stranded DNA breaks

178
Q

Etoposide Indication (3)

A
  1. Testicular CA
  2. Lymphoma
  3. SOLC
179
Q

Etoposide SE

A

Leukomogenic (Leukemia Promoter)

180
Q

Ganciclovir has a severe SE of ____ that is exacerbated with what 2 drugs?

A

Ganciclovir has a severe SE of Neutrophenia ; worsened with

Zidovudine

or

Bactrim

181
Q

Celecoxib MOA

A

Selective COX2 only inhibitor –> DEC inflammation/pain

platelet aggregation and GI protection remain intact

182
Q

Function of Ristocetin

A

Activates [GP1B Platelet R] to make them available for vWF to adhere them to exposed subendothelial collagen

183
Q

Sickle Cell Anemia Clinical Presentation (3)

A
  1. Exeritional Dyspnea
  2. PNA –> Acute Chest Syndrome
  3. Bone Pain
184
Q

Sickle Cell Anemia Genetic MOD

A

point mutation –> Valine being substituted for Glutamic acid in the 6th position of the [HgB B chain]

185
Q

Which enzyme deficiency of Heme Synthesis actually involves Photosensitivity and what is this called? and how will this manifest?

A

[UROD - UROPORPHYRINOGEN DECARBOXYLASE deaminiase] deficiency –> [Porphyria Cutanea Tarda]

Photosensitivity –> [Vesicle & Blister formation] in sun-expoed areas

186
Q

How does [Porphyria Cutanea Tarda] manifest?

A

Photosensitivity –> [Vesicle & Blister formation] in sun-expoed areas (not seen in Acute intermittent porphyria)

[Porphyria Cutanea Tarda] = Uroporphyrinogen Decarboxylase Deficiency or UROD

187
Q

[EBRT - External Beam Radiation Therapy] MOD (2)

A

ionizing radiation causes Double stranded DNA breaks and formation of O2 Free Radicals

188
Q

How is [Pyruvate Kinase Deficiency] related to Red Pulp Hyperplasia

A

PK Deficiency –> Failure of Glycolysis–>No ATP to maintain RBC structure–> OVERWORK from spleen to remove the deformed RBC –> Splenomegaly = Red Pulp Hyperplasia

189
Q

What is the surface marker for Macrophages

A

CD14

190
Q

How does [inhaled amyl nitrite] treate Cyanide Poisoning

A

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
Q

Cyanide Poisoning Tx (3)

A
  1. [Inhaled Amyl Nitrite]
  2. HydroxyCoBalamin (VitB12 precursor) - excretes Cyanide in urine
  3. [Na+ Thiosulfate] -excretes Cyanide in urine
192
Q

Describe the Process of Heme –> Brown Feces (6)

A