Hematology and Oncology Flashcards

1
Q

which platelet granules contain ADP and calcium

A

dense granules

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2
Q

which platelet granules contain vWF, fibrinogen, and fibronectin

A

alpha granules

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3
Q

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

A

specific granules

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4
Q

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

A

azurophilic granules

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5
Q

what receptor on macrophages binds LPS lipid A

A

CD14

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6
Q

MOA cromolyn sodium

A

prevents mast cell degranulation, for asthma prophylaxis

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7
Q

components of Hb A

A

2 alpha, 2 beta

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8
Q

components of Hb A2

A

2 alpha, 2 delta

elevated in beta thal minor

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9
Q

components of fetal hemoglobin

A

2 alpha, 2 gamma

elevated in beta thal major

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10
Q

components of Hb H

A

4 betas, seen in 3 alpha deletions

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11
Q

components of Hb Barts

A

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

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12
Q

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

A

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

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13
Q

which factors are inactivated by warfarin

A

II, VII, IX, X, C, S

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14
Q

protein C cleaves

A

V, VIII

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15
Q

acanthocyte (spur cell) assoc dz

A

liver disease, abetalipoproteinemia

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16
Q

degmacyte (bite cell) dz assoc

A

G6PD

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17
Q

echinocyte (burr cell) dz assoc

A

ESRD, liver disease, pyruvate kinase deficiency

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

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18
Q

spherocyte dz assoc

A

hereditary spherocytosis, drug- and infection-induced hemolytic anemia

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19
Q

target cell dz assoc

A

HbC disease, asplenia, liver disease, thalassemia

“‘HALT,’ said the hunter to his target”

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20
Q

Heinz body dz assoc

A

G6PD deficiency. precursor the the bite cell

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21
Q

Howell-Jolly bodies dz assoc

A

aslpenia

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22
Q

microcytic anemias

A

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

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23
Q

normocytic, nonhemolytic anemias

A

early iron deficiency, early ACD, aplastic anemia, CKD

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24
Q

normocytic, intrinsic hemolytic anemias

A

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

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25
Q

normocytic extrinsic hemolytic anemias

A

autoimmune, microangiopathic, macroangiopathic, infections

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26
Q

megaloblastic anemias

A

folate deficiency, B12 deficiency, orotic aciduria

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27
Q

macrocytic, non-megaloblastic anemias

A

liver disease, alcoholism, Diamond-Blackfan anemia

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28
Q

Burton lines

A

lead lines on gingivae

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29
Q

deficiency sideroblastic anemia

A

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

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30
Q

treatment sideroblastic anemia

A

pyridoxine/B6, a cofactor for delta-ALA synthase

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31
Q

which drugs can cause folate deficiency

A

methotrexate, TMP, and phenytoin

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32
Q

defect in orotic aciduria

A

UMP synthase deficiency

if with hyperammonemia, it is ornithin transcarbamylase deficiency

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33
Q

treatment orotic aciduria

A

UMP to bypass UMP synthase deficiency

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34
Q

defect in paroxysmal noturnal hemoglobinuria

A

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

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35
Q

triad of sx in PNH

A

coombs-negative hemolytic anemia, pancytopenia, venous thrombosis

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36
Q

tx PNH

A

eculizumab

37
Q

which dz cause cold agglutinin

A

mycoplasma and mononucleosis

38
Q

threshold neutropenia

A

less than 1500

39
Q

threshold lymphopenia

A

less than 1500 (in kids less than 3000)

40
Q

threshold eosinopenia

A

less than 30

41
Q

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

A

neutrophilia (high)

low eos and lymphs

42
Q

name the intermediates in heme synthesis

A

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

43
Q

enzyme def and location for sideroblastic anemia

A

delta ALA synthase in mitochondria

note this is an XR disease

44
Q

enzyme def and location for lead poisoning

A

1) d-ALA dehydratase in cytoplasm

2) ferrocheletase in mitochondria

45
Q

enzyme def and location for acute intermittent porphyria

A

porphobilinogen deaminase in cytoplasm

46
Q

enzyme def and location for porphyria cutanea tarda

A

uroporphyrinogen decarboxylase in cytoplasm

47
Q

what accumulates in lead poisoning

A

protoporphyrins and delta-ALA

48
Q

what accumulates in AIP

A

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

49
Q

what accumulates in porphyria cutanea tarda

A

uroporphyrin

50
Q

blistering cutaneous photosensitivity is a sx of which porphyria

A

PCT

51
Q

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

A

AIP

52
Q

inheritance for each hemophilia

A

A XR 8
B XR 9
C AR 11

53
Q

MOA desmopressin for blood disorders and use

A

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

note: not effective for hemophilia B

54
Q

acquired cause of antithrombin III deficiency

A

proteinuira

55
Q

cytology for reed sternberg cells

A

CD15+, CD30+

56
Q

translocation and gene Burkitt

A

8;14 c-myc, heavy ig

57
Q

translocation and gene follicular lymphoma

A

14;18 heavy ig, bcl-2

58
Q

translocation and gene mantle cell lymphoma

A

11;14 cyclin d1, heavy Ig

59
Q

translocation and gene CML

A

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

60
Q

translocation and gene APL

A

15;17 PML-RARA

61
Q

translocation ALL with better prognosis

A

12;21

62
Q

T cell type mycosis fungoides

A

CD4

63
Q

cytology ALL

A

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

64
Q

cytology CLL

A

CD20+, CD5+

65
Q

which leukemia/lymphoma is TRAP+

A

hair cell leukemia

66
Q

CML has (high/low) LAP

A

very low

67
Q

benign reactive neutrophlia has (high/low) LAP

A

high

68
Q

translocation and gene Ewing sarcoma

A

11;22 EWS-FLI 1

69
Q

presentations langerhans cell histiocytosis

A

lytic bone lesions and skin rash

or, recurrent otitis media with mastoid mass

70
Q

cytology langerhans cell histiocytosis

A

S100+, CD1a+

71
Q

Birbeck granules

A

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

72
Q

what do you use to monitor warfarin

A

PT

73
Q

what do you use to monitor heparin

A

PTT

74
Q

class of drugs: enoxaparin, dalteparin

A

LMWH

75
Q

class of drugs: bivalirudin

A

direct thrombin inhibitor

76
Q

class of drugs: cilostazol, dipyridamole

A

PDE III inhibitors

77
Q

MOA PDE III inhibitors

A

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

78
Q

MOA eptifibatide, tirofiban

A

GP-IIb/IIIa inhibitors

79
Q

cell cycle independent chemo

A

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

80
Q

MOA cladribine

A

purine analog antimetabolite. causes nephrotoxicity and neurotoxicity

81
Q

MOA cytarabine (arabinofuranosyl cytidine)

A

pyrimidne analog antimetabolite

82
Q

effect of leucovorin on 5FU AEs

A

worsens myelosupression

83
Q

effect of leucovorin on methotrexate AEs

A

reverses myelosuppression

84
Q

etoposide, teniposide are for topo [I or II]

A

II

85
Q

irinotecan, topotecan are for topo [I or II]

A

I

86
Q

MOA erlotinib

A

EGFR TKI for non-small cell lung carcinoma

87
Q

MOA cetuximab

A

mAb against EGFR

used for stage IV CRC (wildtype KRAS)

88
Q

administer this compound to reduce doxorubicin cardiotox

A

dexrazoxane

89
Q

use this to mitigate cisplatin-induced neprhotoxicity

A

amifostine, a scavenger of free radicals