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Flashcards in Heme/Onc Deck (74):
1

Pure- Red Cell Aplasia (PRCA)

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

2

CO Binding Heme

Competitively binds to heme (not covalent)

3

Hepcidin

Acute phase reactant made in liver that regulates iron
- Low levels stimulate iron absorption in intestine and iron release from macrophages

4

Iron Deficiency

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

5

APL

(t15;17)
- may pw/ hemorrhagic signs and symptoms and persistent infections
- will have immature blast cells and Auer rods

6

Vitamin needed for Carboxylating Glutamate

- VitK needed to carboxylate glutamate to make clotting factors
- Biotin (B7): carboxylation

7

Diseases aw/ JAK mutation (constitutive):

Essential thrombocytopenia
Polycythemia vera
Primary myelofibrosis
(Not CML)

8

Citrate in Preserved Blood

Chelates Ca and Mg
May cause paresthesias

9

Late Defects in Heme Synthesis

(after PBG)
- photosensitivity

10

Early Defects in Heme Synthesis

- neuropsych changes without photosensitivity

11

Bleeding Problems w/ Cystic Fibrosis Due to:

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

12

PTT

Left side: PTT (intrinsic); affected by heparin
- Monitor with aPTT

13

PT

Right side: PT (extrinsic); affected by warfarin
- Monitor PT or INR

14

Glanzmann

gpIIb/IIIa problem (aggregation)

15

Bernard-Soulier

gpIb problem (binding to vwf)

16

Aminocaproic Acid

tPA or alteplase toxicity reversal

17

Warfarin Reversal

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

18

Protamine sulfate

Heparin reversal

19

Homan's Sing

pain that increases with dorsiflexion → indicates a DVT

20

When Iron is Low, Serum Transferrin is _____

increased

21

Porphyrin Synthesis: Early vs Late Defects

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

22

Rat Poison

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

23

Koilonychia

(spoon-shaped nails) with iron deficiency anemia

24

Leukemoid Reaction and CML Differentated By:

Alkaline phosphatase level
- Normal or elevated in leukemoid reaction
- Low in CML

25

Acute Intermittent Porphyria (AIP)

- 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

26

JAK2

Cytoplasmic tyrosine kinase aw/ polycythemia vera

27

Severe anemia (erythroblastosis fetalis) → ___________

release of nucleated RBCs and extramedullary hematopoiesis

28

Migratory Thrombophlebitis

- should always consider cancer
- eg. Pancreatic cancer

29

Prostacyclin (PGI2)

- from endothelial cells
- inhibits platelet aggregation

30

Orotic Aciduria

- Megaloblastic anemia
- treat with uridine to bypass the mutated step
- UMP Synthase defect (build up orotic acid)

31

Angiogenesis Stimulated by:

VEGF and FGF

32

Follicular Lymphoma: Progression

indolent, wax and wane

33

Conversion of Heme to Biliverdin by:

Heme oxygenase

34

HbA2 elevated in:

Beta Thalassemia Minor

35

ALL

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

36

Burkitt Lymphoma

(EBV): jaw (African variant) or abdominal (endemic)
- High mitotic index; round blue small cells
- Starry sky
- C-myc

37

GPIIb/IIIa Direct Inhibitors

Abciximab, Eptifibatide
- blocks a surface receptor that belongs to the integrin family

38

Inhibitors of ADP induced expression of GpIIb/IIIa

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

39

ESR decreased with:

sickle cell, polycythemia, and CHF

40

Heinz bodies

- Hemoglobin precipitation
- G6PD and a-thalassemia

41

Howell-Jolly Bodies

- nuclear remnants
- asplenia/hyposplenia or megaloblastic anemia

42

Pappenheimer Bodies

Iron aggregates/inclusions

43

Burr Cells

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

44

Speed of Hb on Electrophoresis

HbA > HbS > HbC

45

HbA2 elevated in:

B-Thalassemia

46

Drugs that cause AIP (acute intermittent porphyria)

Phenobarbital, sulfonamides, steroid hormones (esp. progresterone

47

_____ & ______ inhibit ALAS (rate-limiting)

Glucose and Heme

48

Effects on Bleeding due to ESRD

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

49

Acquired Antithrombin Deficiency

Loss of antithrombin in urine with nephrotic syndrome or renal failure

50

Tx for CML

Imatinib (Gleevec) Tyrosine Kinase inhibitor

51

Tx for Hairy Cell

Cladribine (adenosine analog)

52

Lytic Bone Lesions seen in:

Multiple Myeloma, ATLL & Langerhan's Histiocytosis

53

Direct Thrombin Inhibitors (4)

Dabigatran, argatroban, bivalirudin, lepirudin

54

Heparin Induced Thrombocytopenia (HIT)

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

55

Cilostazol, Dipyridamole

PDE III inhibitor (increases camp) and inhibits platelet aggregation

56

Etoposide, Teniposide

Inhibits topo II
- used for solid tumors (small cell and testicular), leukemia/lymphoma
- myelosuppression

57

Irinotecan, Topotecan

Inhibits topo I
- colon cancer (irino)
- ovarian and small cell (topotecan)

58

Trastuzumab: Side effect

Cardiotoxicity

59

Drugs that increase risk of PML

Natalizumab and Rituximab

60

CO poisoning

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

61

Sickle Cell Vitamin Deficiencies

Predisposed to folic acid deficiency due to high cell turnover

62

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

Steroid abuse

63

Acute Hemolytic Transfusion Reaction

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

TdT+

ALL (B-ALL and T-ALL)

65

Transferrin in Iron Deficiency

Transferrin synthesis increases in states of low iron and TIBC increases

66

Biochemical Pathway that uses: Glycine and Succinyl-CoA

Part of the Heme Synthesis Pathway

67

ITP Immune Thrombocytopenic Purpura

immune-mediated consumption of platelets
- low platelet count and petechiae
- bone marrow biopsy results demonstrate that the body has appropriately increased platelet production

68

TTP Thrombotic Thrombocytopenic Purpura

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

69

Petechiae vs Purpura vs Ecchymoses

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

70

PO2 normal, O2 Saturation decreased, O2 content decreased

CO Poisoning

71

PO2 normal, O2 Saturation normal, O2 content increased

Polycythemia

72

PO2 normal, O2 saturation normal, O2 content decreased

Anemia/Chronic blood loss

73

DIC aw/ which Leukemia

APL

74

MC type of Hodgkins Lymphoma

Nodular sclerosis