HemOnc Flashcards

(106 cards)

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
hypercoagulable state causes
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)
26
gas embolus causes
benz/caisson(nitrogen precipitates on rapid rise), surgery
27
anemia classification
microcytic(MCV100)h
28
hypersegmented neutrophil disease
b12/folate deficiency
29
high band(immature) neutrophil condition
myeloid proliferation(infx/CML)
30
M0 activation&surface marker
IFN-gamma, CD14(apc for MHCII)
31
eosinophilia causes
NAACP(neoplasm, asthma, allergy, collagen vascular disease, parasites-helminths)
32
drug preventing mast cell degranulation
cromolyn sodium
33
lymphocyte markers
B-CD19, 20. T-CD3, h=4, c=8, regulatory=28
34
warfarin MOA
inhibits epoxide reductase(reduces vitK to active form)
35
clopidigrel/ticlodipine&abciximab MOAs
block ADP receptor, block GpIIb/IIIa
36
microcytic anemias
iron deficiency, chronic disease, sideroblastic (protoporphyin), thallessemia
37
iron transport pathway
enterocyte ferroportin in duodenum, transferrin in blood(TIBC), stored as ferritin
38
bacterial causes of iron deficiency
necatur&ancylostoma(hookworms)
39
iron deficiency labs
microcytic hypochromic(initially normocytic), high TIBC, FEP(proto porphyrin no bound to iron)&RDW(wide range of cell size), low Fe, ferritin, %sat
40
plummer vinson
iron deficiency anemia+esophageal web+beefy red tongue
41
chronic disease anemia mechanism
hepcidin released(sequesters iron+suppress EPO)
42
chronic disease anemia labs
high ferritin/FEP, low Fe/%sat/TIBC
43
protoporphyin synthesis
succinylcoA to ALA(B6) to porphobilinogen plus iron=heme in mitochondria
44
characteristic cell of sideroblastic anemia
ring sideroblast(in bone marrow in mitochondria)
45
sideroblastic anemia causes
congenital(ALAS), alcohol, lead, B6 deficiency(isoniazid)
46
sideroblastic labs
high Fe, ferritin, %sat, low TIBC
47
thalessemia protection
plasmodium falciparum malaria
48
thalassemia genes
alpha-deletion 4 allelles chromosome 16, beta-mutaion 2 allelles chromosome 11
49
alpha thalessemia stages
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
beta thalessemia types
minor(1B+)-mild anemia increased RBC&HbA2, major-(B0B0)-severe months after birth, alpha tetramers
51
beta thalassemia majors pathology
masssive erythroid hyperplasia-crew cut, chipmunk skull, extramedullary hematopoesis, target cells+nucleated cells, no HbA
52
macrocytic anemia causes
B12/folate deficiency, alcohol, liver disease, 5FU
53
folate absorption+ labs
jejunum(green veggies)-high homocysteine, normal MMA
54
B12 deficiency absorption
with IF(loss=pernicious anemia), pancreatic insufficiency, ileum damage(crohns/diphyllabatum)
55
B12 labs
high MMA(spinal cord systems), high homocysteine
56
normocytic anemia labs
reticulocytes(blue RNA)*Hct/45->3%=normal bone marrow(hemolysis)
57
extravascular vs intravascular hemolysis
reticuloendothelial(spleen liver lymph) vs. blood cells
58
extravascular hemolysis findings
anemia, splenomegaly, unconjugated bili(jaundice)
59
intravascular hemolysis findings
low free haptoglobin(bind Hb), Hb/hemosiderin uria/emia
60
hereditary spherocytosis biochemistry
spectrin/ankyrin/band 3.1 deficiency=cytoskeletin tether
61
parvo B19 infection sequelae in anemia
aplastic crises
62
hereditary spherocytosis labs/Tx
round, high RDW/MCHC, osmotic fragility test. Tx-splenectomy
63
sickle cell anemia genes
autoR 2beta globin Val for glutamic acid(HbS-polymerizes under deoxygenation)
64
protective vs polymerizing agents
HbF protects(OHurea increases), acidosis/hypoxia/ dehydration
65
sickle cell pathology
massive erythroid hyperplasia(like thalassemia), vaso-occlusion(swollen hands infant, autosplenectomy, acute chest syndrome-precipitated by pneumonia)
66
removed spleen dysfunctions
capsule infx, salmonella paratyphi, howell jolly bodies
67
sickle cell trait
1 normal 1 beta, microscopic hematuria of kidney medulla. metabisulfite screen
68
hemoglobin c mutation& pathology
autoR lysine for glutamic acid in beta, crystals
69
paroxysmal noctural hemoglobinuria gene
acquired defect in myeloid GPI(no DAF/MIRL-susceptable to complement)
70
paroxysmal nocturnal hemoglobinuria test & association
sucrose& acidify(both activate complement), lack of CD55 on RBC(DAF protein). AML
71
G6PD deficiency gene
autoR decrease 1/2life(african-mild, mediteranean severe)
72
G6PD pathology/lab
oxidative stress=heinz bodies, bite cells. Check heinz prep/enzymes
73
autoimmune hemolytic anemia associations/pathology
IgG=CLL, SLE, penicillin/mdopa, spherocytosis. IgM=cold agglutinin, mycoplasma/mono
74
autoimmune hemolytic anemia tests
direct(IgG antibodies bound)/indirect coombs(serum antibodies)
75
microangiopathic hemolytic anemia pathology
schistocytes(from TTP/HUS/DIC/HELLP/aortic stenosis/prosthetic valves)
76
malaria microbe/bug&infection sites
anopheles mosquito, plasmodium falciparum(daily fever)/ ovale&vivax(fever every other day), liver&RBCs
77
CLL findings
B cells CD5,20, smudge cells. low gamma globulin, can transform to large b cell lymphoma
78
CML
granulocyte increase BASOPHILIA(9,22) bcr-abl TK, Tx-imatanib(blocks TK) can transform to AML/ALL
79
lymphocyte proliferation infection
viral&bordatella
80
EBV findings/test
paracortex(marrow)PALS(spleen)CD8tcells expansion. monospot-IgM heterophile antibodies(negative=CMV)
81
leukemia definition
>20% blasts(crowd out other cell types)
82
AML vs ALL
TdT(DNA pol)=lymph, myelo=myeloperxidase(auer rods)
83
ALL associations &BvsT
downs after 5, B=CD10,19,20(12,21 gene=good, 9,22=poor). T=CD3-8
84
AML subtypes
promyelo-15,17(disrupted RAR, DIC risk, Tx-alltransRA) monocytic(gums) megakaryblastic(downs before 5)
85
chronic leukemia hallmark
high WBC
86
hairy cell labs/Tx
hairy cells, TRAP, red pulp in spleen. Tx-2CDA(adenosine deaminase)
87
ATLL association
CD4, HTLV-1. lytic bone lesions w/high Ca&rash
88
mycosis fungoides
epidermal t cells(pautrier microabscesses)
89
sezary syndrome
cerebriform nuclei in blood
90
polycythemia vera/essential thrombocythemia/ myelofibrosis gene
JAK2 kinase(pv-flushing/itching, et-bleed/thrombi, mf-high PDGF/teardrop cells)
91
follicular lymphoma genes&Tx
(14,18)bcl2-inhibit apoptosis, CD20, small B cells, rituximab
92
mantle zone lymphoma genes
(11, 14) cycline D1-G1 to S phase.
93
marginal zone lymphoma associations
Sjogrens, hashimotos, H pylori infx (MALToma=mucosal)
94
burkitt lymphoma associations/genes
EBV, medium B cells, (8,14) cmyc, starry sky
95
diffuse large cell B lymphoma associations
diffuse sheets
96
hodgkin lympoma characteristics
reed sternberg cell(CD15 CD30)owl eyes, secrete cytokines
97
most common bone malignancy
multiple myeloma
98
multiple myeloma genes/associations
plasma cells, high IL5, RANK receptor, punched out bone
99
multiple myeloma labs
IgG M spike, bence jones proteins(light chain) in urine, deposition in kidney(myeloma kidney), high Ca/amyloid
100
M spike with no myeloma signs
MGUS(1% progress to myeloma)
101
High IgM
B cell lymphoma waldenstrom macroglobulinemia
102
langerhans cell histiocytosis labs
birbeck(tennis racket granules), CD1a/S100+
103
langerhans cell proliferation diseases
letterer-siwe(malignant, rash, fatal), eosinophilic granuloma(benign, bone no skin), hand-schuller-christian (malignant, skull, DI, exopthalmos)
104
main porphyrias/enzyme affected
lead poisening-ALA, acute intermittent-porphobilinogen, porphyria cutanea tarda-uroporphyrin(common, tea urine)
105
GPIIa/IIIb inhibiting drugs
tirofiban, eptifibatide, abciximab
106
sickle cell ostomyelitis bacteria
Staph aureus(normal most common), salmonella