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HemOnc Flashcards

1
Q

Hemophilia A/B labs

A

increased PTT(factor VIII/IX intrinsic deficiency) Xrec

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2
Q

vWD labs

A

increased bleeding time+PTT(autoD)

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3
Q

vWD test

A

ristocetin

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4
Q

vWD Tx

A

desmopressin(increases release of vWF)

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5
Q

pure red cell aplasia association

A

thymic tumors

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6
Q

pure red cell aplasia association

A

thymic tumors

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7
Q

CML genetics

A

Philadelphia chromosome(9,22), BCR-ABL gene, negative ALP&myeloperoxidase

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8
Q

CML Tx

A

imatinib

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9
Q

endothelin effect

A

vasoconstrict immediately post vessel injury

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10
Q

platelet binding, activating&aggregation molecules

A

vWF, ADP(activate IIb/IIIa=fibrinogen), TXA2

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11
Q

most common cause of thrombocytopenia

A

ITP(IgG against platelet antigens) SLE association, Tx-CS/splenectomy

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12
Q

microangiopathic hemolytic anemia types/characteristic cell

A

TTP/HUS, schistocytes

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13
Q

TTP dysfunction

A

ADAMTS13 deficiency(normally cleave vWF)

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14
Q

HUS specific bug

A

Ecoli 0157:H7-undercooked beef, verotoxin endothelial damage

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15
Q

bernard-soulier deficiency

A

Gp1B deficiency(platelet can’t adhere) large platelets

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16
Q

glanzmans thrombasthenia

A

IIb/IIIa deficiency(no aggregation)

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17
Q

intrinsic clot pathway test,factors, drug

A

PTT-12,11,9, 8-subendothelial collagen activates, heparin

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18
Q

extrinsic clot pathway test&factors

A

PT-7-tissue thromboplastin activates, coumadin

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19
Q

vitamin K function

A

gamma carboxylation of factors- II,VII,IX,X, C&S

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20
Q

heparin induced thrombocytopenia pathology

A

PF4 antibodies, thrombosis

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21
Q

DIC causes&test

A

delivery/sepsis/snakebite/myelo leukemia/adenocarcinoma D-Dimer(split fibrin product)

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22
Q

thrombus characteristic predeath

A

lines of zahn/vessel wall attachment

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23
Q

endothelial wall protective molecules

A

PGI2, NO, antithrombin 3, tPA, thrombomodulin

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24
Q

endothelial damage risk factors

A

atherosclerosis, vasculitis, high homocysteine-b12folate deficiency/CBS(convert homocysteine)

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25
Q

hypercoagulable state causes

A

protein c/s deficiency(inactivate V/VIII), factor V leiden (can’t be deactivated by c/s), prothrombin 20210a (point mutation), ATIII deficiency(heparin resistant)

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26
Q

gas embolus causes

A

benz/caisson(nitrogen precipitates on rapid rise), surgery

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27
Q

anemia classification

A

microcytic(MCV100)h

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28
Q

hypersegmented neutrophil disease

A

b12/folate deficiency

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29
Q

high band(immature) neutrophil condition

A

myeloid proliferation(infx/CML)

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30
Q

M0 activation&surface marker

A

IFN-gamma, CD14(apc for MHCII)

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31
Q

eosinophilia causes

A

NAACP(neoplasm, asthma, allergy, collagen vascular disease, parasites-helminths)

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32
Q

drug preventing mast cell degranulation

A

cromolyn sodium

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33
Q

lymphocyte markers

A

B-CD19, 20. T-CD3, h=4, c=8, regulatory=28

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34
Q

warfarin MOA

A

inhibits epoxide reductase(reduces vitK to active form)

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35
Q

clopidigrel/ticlodipine&abciximab MOAs

A

block ADP receptor, block GpIIb/IIIa

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36
Q

microcytic anemias

A

iron deficiency, chronic disease, sideroblastic (protoporphyin), thallessemia

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37
Q

iron transport pathway

A

enterocyte ferroportin in duodenum, transferrin in blood(TIBC), stored as ferritin

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38
Q

bacterial causes of iron deficiency

A

necatur&ancylostoma(hookworms)

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39
Q

iron deficiency labs

A

microcytic hypochromic(initially normocytic), high TIBC, FEP(proto porphyrin no bound to iron)&RDW(wide range of cell size), low Fe, ferritin, %sat

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40
Q

plummer vinson

A

iron deficiency anemia+esophageal web+beefy red tongue

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41
Q

chronic disease anemia mechanism

A

hepcidin released(sequesters iron+suppress EPO)

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42
Q

chronic disease anemia labs

A

high ferritin/FEP, low Fe/%sat/TIBC

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43
Q

protoporphyin synthesis

A

succinylcoA to ALA(B6) to porphobilinogen plus iron=heme in mitochondria

44
Q

characteristic cell of sideroblastic anemia

A

ring sideroblast(in bone marrow in mitochondria)

45
Q

sideroblastic anemia causes

A

congenital(ALAS), alcohol, lead, B6 deficiency(isoniazid)

46
Q

sideroblastic labs

A

high Fe, ferritin, %sat, low TIBC

47
Q

thalessemia protection

A

plasmodium falciparum malaria

48
Q

thalassemia genes

A

alpha-deletion 4 allelles chromosome 16, beta-mutaion 2 allelles chromosome 11

49
Q

alpha thalessemia stages

A

deletion of genes-1-asymptomatic, 2-mild anemia slightly increase RBC-cis worse than trans, 3-severe anemia-HBH tetramers, 4-hydrops fetalis-fatal gamma tetramers

50
Q

beta thalessemia types

A

minor(1B+)-mild anemia increased RBC&HbA2, major-(B0B0)-severe months after birth, alpha tetramers

51
Q

beta thalassemia majors pathology

A

masssive erythroid hyperplasia-crew cut, chipmunk skull, extramedullary hematopoesis, target cells+nucleated cells, no HbA

52
Q

macrocytic anemia causes

A

B12/folate deficiency, alcohol, liver disease, 5FU

53
Q

folate absorption+ labs

A

jejunum(green veggies)-high homocysteine, normal MMA

54
Q

B12 deficiency absorption

A

with IF(loss=pernicious anemia), pancreatic insufficiency, ileum damage(crohns/diphyllabatum)

55
Q

B12 labs

A

high MMA(spinal cord systems), high homocysteine

56
Q

normocytic anemia labs

A

reticulocytes(blue RNA)*Hct/45->3%=normal bone marrow(hemolysis)

57
Q

extravascular vs intravascular hemolysis

A

reticuloendothelial(spleen liver lymph) vs. blood cells

58
Q

extravascular hemolysis findings

A

anemia, splenomegaly, unconjugated bili(jaundice)

59
Q

intravascular hemolysis findings

A

low free haptoglobin(bind Hb), Hb/hemosiderin uria/emia

60
Q

hereditary spherocytosis biochemistry

A

spectrin/ankyrin/band 3.1 deficiency=cytoskeletin tether

61
Q

parvo B19 infection sequelae in anemia

A

aplastic crises

62
Q

hereditary spherocytosis labs/Tx

A

round, high RDW/MCHC, osmotic fragility test. Tx-splenectomy

63
Q

sickle cell anemia genes

A

autoR 2beta globin Val for glutamic acid(HbS-polymerizes under deoxygenation)

64
Q

protective vs polymerizing agents

A

HbF protects(OHurea increases), acidosis/hypoxia/ dehydration

65
Q

sickle cell pathology

A

massive erythroid hyperplasia(like thalassemia), vaso-occlusion(swollen hands infant, autosplenectomy, acute chest syndrome-precipitated by pneumonia)

66
Q

removed spleen dysfunctions

A

capsule infx, salmonella paratyphi, howell jolly bodies

67
Q

sickle cell trait

A

1 normal 1 beta, microscopic hematuria of kidney medulla. metabisulfite screen

68
Q

hemoglobin c mutation& pathology

A

autoR lysine for glutamic acid in beta, crystals

69
Q

paroxysmal noctural hemoglobinuria gene

A

acquired defect in myeloid GPI(no DAF/MIRL-susceptable to complement)

70
Q

paroxysmal nocturnal hemoglobinuria test & association

A

sucrose& acidify(both activate complement), lack of CD55 on RBC(DAF protein). AML

71
Q

G6PD deficiency gene

A

autoR decrease 1/2life(african-mild, mediteranean severe)

72
Q

G6PD pathology/lab

A

oxidative stress=heinz bodies, bite cells. Check heinz prep/enzymes

73
Q

autoimmune hemolytic anemia associations/pathology

A

IgG=CLL, SLE, penicillin/mdopa, spherocytosis. IgM=cold agglutinin, mycoplasma/mono

74
Q

autoimmune hemolytic anemia tests

A

direct(IgG antibodies bound)/indirect coombs(serum antibodies)

75
Q

microangiopathic hemolytic anemia pathology

A

schistocytes(from TTP/HUS/DIC/HELLP/aortic stenosis/prosthetic valves)

76
Q

malaria microbe/bug&infection sites

A

anopheles mosquito, plasmodium falciparum(daily fever)/ ovale&vivax(fever every other day), liver&RBCs

77
Q

CLL findings

A

B cells CD5,20, smudge cells. low gamma globulin, can transform to large b cell lymphoma

78
Q

CML

A

granulocyte increase BASOPHILIA(9,22) bcr-abl TK, Tx-imatanib(blocks TK) can transform to AML/ALL

79
Q

lymphocyte proliferation infection

A

viral&bordatella

80
Q

EBV findings/test

A

paracortex(marrow)PALS(spleen)CD8tcells expansion. monospot-IgM heterophile antibodies(negative=CMV)

81
Q

leukemia definition

A

> 20% blasts(crowd out other cell types)

82
Q

AML vs ALL

A

TdT(DNA pol)=lymph, myelo=myeloperxidase(auer rods)

83
Q

ALL associations &BvsT

A

downs after 5, B=CD10,19,20(12,21 gene=good, 9,22=poor). T=CD3-8

84
Q

AML subtypes

A

promyelo-15,17(disrupted RAR, DIC risk, Tx-alltransRA) monocytic(gums) megakaryblastic(downs before 5)

85
Q

chronic leukemia hallmark

86
Q

hairy cell labs/Tx

A

hairy cells, TRAP, red pulp in spleen. Tx-2CDA(adenosine deaminase)

87
Q

ATLL association

A

CD4, HTLV-1. lytic bone lesions w/high Ca&rash

88
Q

mycosis fungoides

A

epidermal t cells(pautrier microabscesses)

89
Q

sezary syndrome

A

cerebriform nuclei in blood

90
Q

polycythemia vera/essential thrombocythemia/ myelofibrosis gene

A

JAK2 kinase(pv-flushing/itching, et-bleed/thrombi, mf-high PDGF/teardrop cells)

91
Q

follicular lymphoma genes&Tx

A

(14,18)bcl2-inhibit apoptosis, CD20, small B cells, rituximab

92
Q

mantle zone lymphoma genes

A

(11, 14) cycline D1-G1 to S phase.

93
Q

marginal zone lymphoma associations

A

Sjogrens, hashimotos, H pylori infx (MALToma=mucosal)

94
Q

burkitt lymphoma associations/genes

A

EBV, medium B cells, (8,14) cmyc, starry sky

95
Q

diffuse large cell B lymphoma associations

A

diffuse sheets

96
Q

hodgkin lympoma characteristics

A

reed sternberg cell(CD15 CD30)owl eyes, secrete cytokines

97
Q

most common bone malignancy

A

multiple myeloma

98
Q

multiple myeloma genes/associations

A

plasma cells, high IL5, RANK receptor, punched out bone

99
Q

multiple myeloma labs

A

IgG M spike, bence jones proteins(light chain) in urine, deposition in kidney(myeloma kidney), high Ca/amyloid

100
Q

M spike with no myeloma signs

A

MGUS(1% progress to myeloma)

101
Q

High IgM

A

B cell lymphoma waldenstrom macroglobulinemia

102
Q

langerhans cell histiocytosis labs

A

birbeck(tennis racket granules), CD1a/S100+

103
Q

langerhans cell proliferation diseases

A

letterer-siwe(malignant, rash, fatal), eosinophilic granuloma(benign, bone no skin), hand-schuller-christian (malignant, skull, DI, exopthalmos)

104
Q

main porphyrias/enzyme affected

A

lead poisening-ALA, acute intermittent-porphobilinogen, porphyria cutanea tarda-uroporphyrin(common, tea urine)

105
Q

GPIIa/IIIb inhibiting drugs

A

tirofiban, eptifibatide, abciximab

106
Q

sickle cell ostomyelitis bacteria

A

Staph aureus(normal most common), salmonella

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