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Flashcards in Hematology and Oncology Deck (89):
1

which platelet granules contain ADP and calcium

dense granules

2

which platelet granules contain vWF, fibrinogen, and fibronectin

alpha granules

3

which neutrophil granules contain LAP, collagenase, lysozyme, and lacteferrin

specific granules

4

which neutrophil granules contain proteinases, acid phosphatase, MPO, and beta-glucuronidase

azurophilic granules

5

what receptor on macrophages binds LPS lipid A

CD14

6

MOA cromolyn sodium

prevents mast cell degranulation, for asthma prophylaxis

7

components of Hb A

2 alpha, 2 beta

8

components of Hb A2

2 alpha, 2 delta
elevated in beta thal minor

9

components of fetal hemoglobin

2 alpha, 2 gamma
elevated in beta thal major

10

components of Hb H

4 betas, seen in 3 alpha deletions

11

components of Hb Barts

4 gamma, seen in 4 alpha deletions which causes fatal hydrops fetalis

12

which hemoglobin moves the farthest on electrophoresis, and which are behind

A goes the farthest, then F, then S, then C

13

which factors are inactivated by warfarin

II, VII, IX, X, C, S

14

protein C cleaves

V, VIII

15

acanthocyte (spur cell) assoc dz

liver disease, abetalipoproteinemia

16

degmacyte (bite cell) dz assoc

G6PD

17

echinocyte (burr cell) dz assoc

ESRD, liver disease, pyruvate kinase deficiency

note projections are more uniform and smaller than those of the spur cell

18

spherocyte dz assoc

hereditary spherocytosis, drug- and infection-induced hemolytic anemia

19

target cell dz assoc

HbC disease, asplenia, liver disease, thalassemia

("'HALT,' said the hunter to his target")

20

Heinz body dz assoc

G6PD deficiency. precursor the the bite cell

21

Howell-Jolly bodies dz assoc

aslpenia

22

microcytic anemias

late iron deficiency, late ACD, thalassemias, lead poisoning, sideroblastic anemia

23

normocytic, nonhemolytic anemias

early iron deficiency, early ACD, aplastic anemia, CKD

24

normocytic, intrinsic hemolytic anemias

RBC membrane defects (e.g. HS), enzyme defects (e.g. G6PD and PK), HbC disease, sickle cell, PNH

25

normocytic extrinsic hemolytic anemias

autoimmune, microangiopathic, macroangiopathic, infections

26

megaloblastic anemias

folate deficiency, B12 deficiency, orotic aciduria

27

macrocytic, non-megaloblastic anemias

liver disease, alcoholism, Diamond-Blackfan anemia

28

Burton lines

lead lines on gingivae

29

deficiency sideroblastic anemia

XR in delta-ALA synthase, which is first step and RLS (occurs in mitochondria)

30

treatment sideroblastic anemia

pyridoxine/B6, a cofactor for delta-ALA synthase

31

which drugs can cause folate deficiency

methotrexate, TMP, and phenytoin

32

defect in orotic aciduria

UMP synthase deficiency

if with hyperammonemia, it is ornithin transcarbamylase deficiency

33

treatment orotic aciduria

UMP to bypass UMP synthase deficiency

34

defect in paroxysmal noturnal hemoglobinuria

defect in CD55 (decay-accelerating factor) and/or CD 59 (MAC-inhibitory protein)

35

triad of sx in PNH

coombs-negative hemolytic anemia, pancytopenia, venous thrombosis

36

tx PNH

eculizumab

37

which dz cause cold agglutinin

mycoplasma and mononucleosis

38

threshold neutropenia

less than 1500

39

threshold lymphopenia

less than 1500 (in kids less than 3000)

40

threshold eosinopenia

less than 30

41

results of steroids on blood levels of eosinophils, lymphocytes, and neutrophils

neutrophilia (high)
low eos and lymphs

42

name the intermediates in heme synthesis

glycine + succinyl-CoA > delta ALA > porphobilinogen > hydroxymethylbilane > uroporphyrinogen III > coproporphyrinogen III > protoporphyrin > heme

43

enzyme def and location for sideroblastic anemia

delta ALA synthase in mitochondria

note this is an XR disease

44

enzyme def and location for lead poisoning

1) d-ALA dehydratase in cytoplasm

2) ferrocheletase in mitochondria

45

enzyme def and location for acute intermittent porphyria

porphobilinogen deaminase in cytoplasm

46

enzyme def and location for porphyria cutanea tarda

uroporphyrinogen decarboxylase in cytoplasm

47

what accumulates in lead poisoning

protoporphyrins and delta-ALA

48

what accumulates in AIP

porphobilinogen, delta-ALA, coporphobilinogen (in the urine. != coproporphyrinogen III)

49

what accumulates in porphyria cutanea tarda

uroporphyrin

50

blistering cutaneous photosensitivity is a sx of which porphyria

PCT

51

abdominal pain, polyneuropathy, port-wine colored urine and psychological distrubances is a sx of which porphyria

AIP

52

inheritance for each hemophilia

A XR 8
B XR 9
C AR 11

53

MOA desmopressin for blood disorders and use

increases vWF release from platelets. good for vWD and hemophilia A

note: not effective for hemophilia B

54

acquired cause of antithrombin III deficiency

proteinuira

55

cytology for reed sternberg cells

CD15+, CD30+

56

translocation and gene Burkitt

8;14 c-myc, heavy ig

57

translocation and gene follicular lymphoma

14;18 heavy ig, bcl-2

58

translocation and gene mantle cell lymphoma

11;14 cyclin d1, heavy Ig

59

translocation and gene CML

9;22. bcr-abl (abl is on 9) also seen in some ALL

60

translocation and gene APL

15;17 PML-RARA

61

translocation ALL with better prognosis

12;21

62

T cell type mycosis fungoides

CD4

63

cytology ALL

TDT+ (pre-T and pre-B), CD10+ (pre-B)

64

cytology CLL

CD20+, CD5+

65

which leukemia/lymphoma is TRAP+

hair cell leukemia

66

CML has (high/low) LAP

very low

67

benign reactive neutrophlia has (high/low) LAP

high

68

translocation and gene Ewing sarcoma

11;22 EWS-FLI 1

69

presentations langerhans cell histiocytosis

lytic bone lesions and skin rash
or, recurrent otitis media with mastoid mass

70

cytology langerhans cell histiocytosis

S100+, CD1a+

71

Birbeck granules

tennis racket-shaped granules seen on EM in langerhans cell histiocytosis

72

what do you use to monitor warfarin

PT

73

what do you use to monitor heparin

PTT

74

class of drugs: enoxaparin, dalteparin

LMWH

75

class of drugs: bivalirudin

direct thrombin inhibitor

76

class of drugs: cilostazol, dipyridamole

PDE III inhibitors

77

MOA PDE III inhibitors

cause increase in platelet cAMP, inhibiting aggregation and causing vasodilation. used in angina prophylaxis

78

MOA eptifibatide, tirofiban

GP-IIb/IIIa inhibitors

79

cell cycle independent chemo

platinum agents (e.g. cisplatin) and alkylating agents (busulfan, cyclophos, ifosfamide, nitrosoureas)

80

MOA cladribine

purine analog antimetabolite. causes nephrotoxicity and neurotoxicity

81

MOA cytarabine (arabinofuranosyl cytidine)

pyrimidne analog antimetabolite

82

effect of leucovorin on 5FU AEs

worsens myelosupression

83

effect of leucovorin on methotrexate AEs

reverses myelosuppression

84

etoposide, teniposide are for topo [I or II]

II

85

irinotecan, topotecan are for topo [I or II]

I

86

MOA erlotinib

EGFR TKI for non-small cell lung carcinoma

87

MOA cetuximab

mAb against EGFR

used for stage IV CRC (wildtype KRAS)

88

administer this compound to reduce doxorubicin cardiotox

dexrazoxane

89

use this to mitigate cisplatin-induced neprhotoxicity

amifostine, a scavenger of free radicals