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Step 1 > Flashcards - HemeOnc > Flashcards

Flashcards - HemeOnc

1
Q

ristocetin test

A

von Willebrand Disease - decreased platelet aggregation

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2
Q

PALS (perarterial lymphoid sheath) contain?

A

T-cells

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3
Q

Paroxysmal Nocturnal Hemoglobinuria

A

PIGA mutation –> unable to synthesize GPI anchor (CD55, CD59) that inactivate complement and prevent MACs - sucrose test can activate complement - treat with eculizumab

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4
Q

Lupus anticoagulant

A

Prolonged PTT, not correctable with FFP

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5
Q

Hereditary Spherocytosis defect

A

ankrin or spectrin

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6
Q

TTP Autoantibody

A

ADAMTS13 (von Willebrand factor metalloprotease)

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7
Q

tartrate-resistant acid phosphatase (TRAP)

A

Hairy Cell Leukemia

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8
Q

EBV infection increases risk of

A

Hodgkin’s disease, nasopharyngeal cancer

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9
Q

Plummer Vinson Triad

A

Iron deficiency anemia, dysphagia, esophageal webs

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10
Q

Cold agglutinin

A

IgM - CLL, mycoplasma pneunomia, mononucleosis,

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11
Q

Warm agglutinin

A

IgG - SLE, CLL, drugs (a-methyldopa)

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12
Q

acute promyelocytic leukemia translocation

A

t(15:17)

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13
Q

chronic myelogenous leukemia translocation

A

t(9:22)

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14
Q

5-flourouracil

A

pyrimidine antimetabolite

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15
Q

cyclophosphamide

A

alkylating agent

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16
Q

cytarabine

A

pyrimidine analog

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17
Q

methotrexate

A

folate antimetabolite

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18
Q

6-mercaptopurine

A

purine nucleotide synthesis inhibitor

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19
Q

Waldenstrom Macroglobulinemia

A

B-cell lymphoma with monoclonal IgM - visual/neuro defects - treat with plasmapheresis

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20
Q

Glanzmann’s thrombastehnia

A

decreased glycoproteins (Gps) IIb/IIIa

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21
Q

Lead poisoning

A

Blocks ALA dehydrogenase and ferrochelatase - increased gamma-aminovulinate dehydratase

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22
Q

ADAMTS13

A

cleaves vWF - inhibition leads to TTP

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23
Q

CA-125

A

ovarian cancer

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24
Q

carcinoembryonic antigen/CEA

A

colorectal cancer

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25
Q

alpha-fetoprotein

A

hepatocellular carcinoma

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26
Q

CA 19-9

A

pancreatic cancer

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27
Q

GI mucosal layers

A

Serosa –> Musclaris (outer, myenteric/Auerbach, inner) –> Submucosa (Meissner, glands) –> Mucosa (musclaris mucosa, lamina propria, epithelium)

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28
Q

Inguinal layers

A

skin, scarpas, fascia, external oblique, internal oblique, transversus, fascia, extraperitoneal fat, peritoneum

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29
Q

Cremaster origin

A

Internal oblique

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30
Q

sheets/nests of uniform cells, oval/round stippled nuclei

A

carcinoid tumor

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31
Q

MIF secretion

A

Sertoli cells

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32
Q

theca cell hormones

A

stimulaton from LH - cholesterol –> progesterone + androgens

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33
Q

granulosa cell hormones

A

stimulation from FSH - androgen –> estrogen

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34
Q

congenital rubella/rubeola infection

A

deafness, cataracts, cardiac malformations

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35
Q

basophilic stippling of hypochromic microcytic RBCs

A

lead poisoning

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36
Q

CML

A

complete spectrum of granulocytic cells, <10% blasts, decreased Alk Phos

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37
Q

AML

A

increased number of myeloblasts >20%

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38
Q

CLL

A

increased mature lymphoid cells

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39
Q

Cladribine

A

purine analog for hairy cell leukemia

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40
Q

Vincristine

A

inhibits microtubule polymerization by binding to B-tubulin, prevents mitotic spindle (side effect of neurotoxicity)

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41
Q

etoposide

A

targets topoisomerase II -induces double stranded DNA breaks

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42
Q

clopidigrel

A

inhibits ADP mediated platelet aggregation

43
Q

argatroban, dabigatran

A

direct thrombin inhibitor

44
Q

enoxaparin

A

low-weight molecular heparin - ATIII activator

45
Q

apixaban, rivaroxaban

A

Factor Xa inhibitor

46
Q

GP IIb/IIIa

A

platelet aggregation protein - attaches to fibrinogen - inhibited by Abciximab - deficient in Glanzmann thrombasthenia, autoantibodies in ITP

47
Q

acute porphyria attack treatment

A

inhibit ALA synthase (a-aminolevulinic acid) with hemin or glucose

48
Q

bite cells

A

G6PD

49
Q

burr cells, helmet cells

A

microangiopathic hemolysis, mechanical damage

50
Q

RBCs that are basophilic on Wright-Giemsa

A

reticulocytes - still have residual ribosomal RNA

51
Q

endothelin

A

vasocontricter

52
Q

Leukemia in Down

A

ALL, acute megakaryoblastic leukemia

53
Q

Sickle cell amino acid

A

Glutamic acid to Valine

54
Q

chloramphenicol adverse effect

A

reversible dose-dependent anemia, leukopenia, thrombocytopenia

55
Q

splenectomy infections

A

Group B strep, H. flu, S. pneumonieae, N. meningitidis, S. typhi

56
Q

leukocyte rolling proteins

A

L-selectin on neutrophils, E/P-selectin on endothelial cells

57
Q

tight adhesion/crawling proteins

A

CD18 beta-2 integrins (Mac-1, LFA-1) to intercellular adhesion molecule-1 (ICAM-1)

58
Q

transmigration protein

A

platelet endothelial cell adhesion molecule 1 (PECAM-1)

59
Q

Weibel-Palade bodies

A

contain vWF and P-selectin

60
Q

polycythemia vera

A

mutations in JAK2 - constitutive kinase activation

61
Q

acute lymphoblastic leukemia

A

usually B-cell (70%), but T-cell can present as mediastinal mass

62
Q

“waxing waning” lymphadenopathy

A

follicular lymphoma (14:18)

63
Q

signet ring carcinoma

A

gastric adenocarcinoma - cells that do not form glands

64
Q

intestinal type adenocarcinoma

A

stomach cancer resembling colon cancer - nodular polypoid - glands/columnar/cuboidal

65
Q

non-rhythmic conjugate eye movements in children

A

neuroblastoma

66
Q

Charcot-Leyden crystals

A

sign of eosinophilic infiltration - allergies

67
Q

Langerhans cell

A

dendritic cell - phagocytic antigen presenting cell - MHC II

68
Q

Protein C action

A

activated protein C + protein S cleaves and inactivates Va and VIIIa

69
Q

acanthocyte

A

“spur cell” - liver disease, abetalipoproteinemia

70
Q

dacrocyte

A

tear drop shaped - bone marrow infiltration (myelofibrosis)

71
Q

Howell Jolly bodies

A

basophilic nuclear remnant normally removed by spleen - can be seen in asplenic pts

72
Q

alpha-thalasssemia

A

4-allele deletion = Hb Barts, hydrops fetalis/death
3-allele deletion = HbH, B4 chains
1-2 allele deletion = less clinically severe anemia

73
Q

orotic aciduria

A

megaloblastic anemia - inability to convert orotic acid to UMP (Uridine monophosphate synthetase) - treat with uridine monophosphate to bypass enzyme

74
Q

HbC defect

A

glutamic acid to lysine mutation in B-globin

75
Q

Bernard-Soulier syndrome

A

Defect in platelet to vWF adhesion - decreased GpIb - no agglutination on ristocetin assay

76
Q

acute intermittent porphyria enzyme

A

porphobilinogen deanimase - accumulates porphobilinogen, delta-ALA, coporphobilinogen - painful abdomen, portwine urine, polyneuropathy, psych, precipitated by drugs/EtOH, starvation

77
Q

porphyria cutanea tarda enzyme

A

uroporphyrinogen decarboxylate - accumulates uroporphyrin - most common porphyria

78
Q

increased bleeding time

A

platelet disorder

79
Q

prothrombin gene mutation

A

hypercoagulability - 3’ untranslated region mutation - increased production of prothrombin

80
Q

reed-sternberg cells CDs

A

CD15 and CD30 B-cell origin

81
Q

CD5+ lymphoma

A

mantle cell t(11:14)

82
Q

adult t-cell lymphoma

A

caused by HTLV - cutaneous lesions, lytic bone lesions, hypercalcemia

83
Q

mycosis fungoides/Sezary syndrome

A

skin patches/plaques (cutaneous T-cell lymphoma), microabscesses - atypical CD4+ cells with “cerebriform” nuclei

84
Q

fried egg/clock faced chromatin on histo, rouleaux formations

A

multiple myeloma

85
Q

monoclonal gammopathy of undertermined significance (MGUS)

A

asymptomatic - 1-2% progress to MM

86
Q

TdT+,

A

ALL - associated with downs

87
Q

TRAP+

A

hairy cell leukemia - treat with cladribine, pentostatin

88
Q

smudge cells

A

CLL - CD20+, CD5+ B-cells - progresses slowly

89
Q

Auer rods

A

AML - M3 subtype is t(15:17)

90
Q

tennis rackets

A

langerhans cell histiocytosis - S100, CD1a

91
Q

LWMH

A

enoxparin, dalteparin - 2-4x longer halflife

92
Q

cilostazol, dipyridamole

A

phosphodiesterase III inhibitor - increases cAMP in platelets, inhibiting aggregation

93
Q

prothrombin factor

A

Factor II - converts fibrinogen to fibrin

94
Q

tPA

A

converts plasminogen to plasmin –> cleaves fibrinogen/fibrin, destroys clotting factors, blocks platelet aggregation

95
Q

radical prostatectomy DIC

A

increased urokinase –> decreased a2-antiplasmin –> degrades fibrinogen with no D-Dimer

96
Q

sideroblastic anemia

A

defect in protoporphyrin synthesis - sideroblast rins - from ALAS mutation, EtOH, lead, B6 def

97
Q

acute monocytic leukemia

A

AML subtype - monoblasts - lacks MPO - infiltrates gums

98
Q

avute megakaryoblastic leukemia

A

AML subtype - lacks MPO - associated with Downs

99
Q

B-ALL

A

most common - TdT+ - CD10,19,20 - prophylactic chemotherapy to scrotum and brain - t(12:21) is good, t(9:22) is bad

100
Q

T-ALL

A

mediastinal mass in teenager (lymphoma) - TdT+ CD2-8, no CD10

101
Q

Letterar-Siwe disease

A

histiocytosis - rash/cystic skeletal defects <2yo, rapidly fatal

102
Q

Hand-Schuller-Christian disease

A

histiocytosis - scalp rash, lytic skin defects, DI, exopthalmos in child >3yo

103
Q

Hodgkin lymphoma prognosis

A

lymphocyte rich = good prognosis, depleted = elderly, HIV, worst prognosis

104
Q

marginal zone lymphoma

A

expands into marginal zone - from chronic inflammatory state or MALToma

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