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Quick Notes for Step 1 > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Pure- Red Cell Aplasia (PRCA)

A

Aw/ Thymic tumors (thymomas), lymphocytic leukemias, and parvovirus B19

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2
Q

CO Binding Heme

A

Competitively binds to heme (not covalent)

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3
Q

Hepcidin

A

Acute phase reactant made in liver that regulates iron

- Low levels stimulate iron absorption in intestine and iron release from macrophages

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4
Q

Iron Deficiency

A

May have glossal pain, dry mouth, loss of taste and alopecia in addition to the weakness, fatigue, headache, etc.

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5
Q

APL

A

(t15;17)

  • may pw/ hemorrhagic signs and symptoms and persistent infections
  • will have immature blast cells and Auer rods
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6
Q

Vitamin needed for Carboxylating Glutamate

A
  • VitK needed to carboxylate glutamate to make clotting factors
  • Biotin (B7): carboxylation
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7
Q

Diseases aw/ JAK mutation (constitutive):

A

Essential thrombocytopenia
Polycythemia vera
Primary myelofibrosis
(Not CML)

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8
Q

Citrate in Preserved Blood

A

Chelates Ca and Mg

May cause paresthesias

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9
Q

Late Defects in Heme Synthesis

A

(after PBG)

- photosensitivity

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10
Q

Early Defects in Heme Synthesis

A
  • neuropsych changes without photosensitivity
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11
Q

Bleeding Problems w/ Cystic Fibrosis Due to:

A

Impaired fat absorption so decreased Vit A, D, E, and K (bleeding problems due to low synthesis of coagulation factors)

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12
Q

PTT

A

Left side: PTT (intrinsic); affected by heparin

- Monitor with aPTT

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13
Q

PT

A

Right side: PT (extrinsic); affected by warfarin

- Monitor PT or INR

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14
Q

Glanzmann

A

gpIIb/IIIa problem (aggregation)

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15
Q

Bernard-Soulier

A

gpIb problem (binding to vwf)

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16
Q

Aminocaproic Acid

A

tPA or alteplase toxicity reversal

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17
Q

Warfarin Reversal

A

Fresh frozen plasma for fastest warfarin reversal, vit K can be used but not as fast

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18
Q

Protamine sulfate

A

Heparin reversal

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19
Q

Homan’s Sing

A

pain that increases with dorsiflexion → indicates a DVT

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20
Q

When Iron is Low, Serum Transferrin is _____

A

increased

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21
Q

Porphyrin Synthesis: Early vs Late Defects

A
  • Early steps: neuropsychiatric manifestations

- Later steps (after PBG): photosensitivity (eg. Acute cutanea tarda with UROD deficiency)

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22
Q

Rat Poison

A

will deplete all vitK dependent coagulation factors and patient should be immediately given fresh frozen plasma

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23
Q

Koilonychia

A

(spoon-shaped nails) with iron deficiency anemia

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24
Q

Leukemoid Reaction and CML Differentated By:

A

Alkaline phosphatase level

  • Normal or elevated in leukemoid reaction
  • Low in CML
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25
Q

Acute Intermittent Porphyria (AIP)

A
  • Urine turns darker (port-wine) with exposure to air/light due to excess PBG
  • No photosensitivity, just abdominal pain
  • HMB synthase deficiency (aka PBG deaminase)
  • Can treat by downregulating ALAS activity with glucose or heme infusion
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26
Q

JAK2

A

Cytoplasmic tyrosine kinase aw/ polycythemia vera

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27
Q

Severe anemia (erythroblastosis fetalis) → ___________

A

release of nucleated RBCs and extramedullary hematopoiesis

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28
Q

Migratory Thrombophlebitis

A
  • should always consider cancer

- eg. Pancreatic cancer

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29
Q

Prostacyclin (PGI2)

A
  • from endothelial cells

- inhibits platelet aggregation

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30
Q

Orotic Aciduria

A
  • Megaloblastic anemia
  • treat with uridine to bypass the mutated step
  • UMP Synthase defect (build up orotic acid)
31
Q

Angiogenesis Stimulated by:

A

VEGF and FGF

32
Q

Follicular Lymphoma: Progression

A

indolent, wax and wane

33
Q

Conversion of Heme to Biliverdin by:

A

Heme oxygenase

34
Q

HbA2 elevated in:

A

Beta Thalassemia Minor

35
Q

ALL

A

MC in children; 70-80% is B-ALL, the rest is T-ALL (mediastinal mass, may have dysphagia, respiratory sx, or SVC syndrome)

36
Q

Burkitt Lymphoma

A

(EBV): jaw (African variant) or abdominal (endemic)

  • High mitotic index; round blue small cells
  • Starry sky
  • C-myc
37
Q

GPIIb/IIIa Direct Inhibitors

A

Abciximab, Eptifibatide

- blocks a surface receptor that belongs to the integrin family

38
Q

Inhibitors of ADP induced expression of GpIIb/IIIa

A

Irreversible: Clopidogrel, Prasugrel, Ticlopidine(neutropenia and mouth ulcers)
Reversible: Ticagrelor

39
Q

ESR decreased with:

A

sickle cell, polycythemia, and CHF

40
Q

Heinz bodies

A
  • Hemoglobin precipitation

- G6PD and a-thalassemia

41
Q

Howell-Jolly Bodies

A
  • nuclear remnants

- asplenia/hyposplenia or megaloblastic anemia

42
Q

Pappenheimer Bodies

A

Iron aggregates/inclusions

43
Q

Burr Cells

A

Seen in microangiopathic hemolytic anemia, mechanical damage or Pyruvate Kinase Deficiency (rigid RBCs)

44
Q

Speed of Hb on Electrophoresis

A

HbA > HbS > HbC

45
Q

HbA2 elevated in:

A

B-Thalassemia

46
Q

Drugs that cause AIP (acute intermittent porphyria)

A

Phenobarbital, sulfonamides, steroid hormones (esp. progresterone

47
Q

_____ & ______ inhibit ALAS (rate-limiting)

A

Glucose and Heme

48
Q

Effects on Bleeding due to ESRD

A

Platelet aggregation is inhibited by uremic toxins –> increased bleeding time

49
Q

Acquired Antithrombin Deficiency

A

Loss of antithrombin in urine with nephrotic syndrome or renal failure

50
Q

Tx for CML

A

Imatinib (Gleevec) Tyrosine Kinase inhibitor

51
Q

Tx for Hairy Cell

A

Cladribine (adenosine analog)

52
Q

Lytic Bone Lesions seen in:

A

Multiple Myeloma, ATLL & Langerhan’s Histiocytosis

53
Q

Direct Thrombin Inhibitors (4)

A

Dabigatran, argatroban, bivalirudin, lepirudin

54
Q

Heparin Induced Thrombocytopenia (HIT)

A

developing IgG against heparing bound to PF4 (platelet factor 4)

55
Q

Cilostazol, Dipyridamole

A

PDE III inhibitor (increases camp) and inhibits platelet aggregation

56
Q

Etoposide, Teniposide

A

Inhibits topo II

  • used for solid tumors (small cell and testicular), leukemia/lymphoma
  • myelosuppression
57
Q

Irinotecan, Topotecan

A

Inhibits topo I

  • colon cancer (irino)
  • ovarian and small cell (topotecan)
58
Q

Trastuzumab: Side effect

A

Cardiotoxicity

59
Q

Drugs that increase risk of PML

A

Natalizumab and Rituximab

60
Q

CO poisoning

A

PaO2 is 95 (dissolved not affected)
Carboxyhemoglobin is increased
Methemoglobin is decreased

61
Q

Sickle Cell Vitamin Deficiencies

A

Predisposed to folic acid deficiency due to high cell turnover

62
Q

Competitive Athlete w/ erythrocytosis, abnormal testicle size, and acne

A

Steroid abuse

63
Q

Acute Hemolytic Transfusion Reaction

A

Antibody mediated (Type II hypersensitivity)
- complement mediated lysis
(different from IgE mediated Type I which would be an allergic transfusion reaction w/ sx of anaphylaxis and urticaria)

64
Q

TdT+

A

ALL (B-ALL and T-ALL)

65
Q

Transferrin in Iron Deficiency

A

Transferrin synthesis increases in states of low iron and TIBC increases

66
Q

Biochemical Pathway that uses: Glycine and Succinyl-CoA

A

Part of the Heme Synthesis Pathway

67
Q

ITP Immune Thrombocytopenic Purpura

A

immune-mediated consumption of platelets

  • low platelet count and petechiae
  • bone marrow biopsy results demonstrate that the body has appropriately increased platelet production
68
Q

TTP Thrombotic Thrombocytopenic Purpura

A 
thrombocytopenia (low platelet count)
microangiopathic hemolytic anemia
altered mental status
renal failure
fever
69
Q

Petechiae vs Purpura vs Ecchymoses

A

Petechiae (less than 5 mm)
Purpura (5mm to 1cm)
Ecchymoses (greater than 1 cm)

70
Q

PO2 normal, O2 Saturation decreased, O2 content decreased

A

CO Poisoning

71
Q

PO2 normal, O2 Saturation normal, O2 content increased

A

Polycythemia

72
Q

PO2 normal, O2 saturation normal, O2 content decreased

A

Anemia/Chronic blood loss

73
Q

DIC aw/ which Leukemia

A

APL

74
Q

MC type of Hodgkins Lymphoma

A

Nodular sclerosis

Quick Notes for Step 1 (18 decks)

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