hem/onc Flashcards

1
Q

acanthocytes?

A

liver disease

abetalipoproteinemia

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2
Q

basophilic stippling?

A

lead poisoning

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3
Q

dacryocyte?

A

= teardrop cell

seen in bone marrow infiltration - e.g. myelofibrosis

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4
Q

Heinz body?

A

Hb precipitation (after oxidation of Hb-SH to -S-S-) in G6PD

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5
Q

Howell-Jolly bodies?

A

basophilic nuclear remnants

in functional hyposplenia or asplenia

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6
Q

fatigue, conjunctival pallor, spoon nails?

A

iron deficiency anemia
block in final step of heme synthesis

** decreased iron, ferritin; increased TIBC (transferrin)

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7
Q

defect in alpha thal?

A

DELETION of alpha globin genes
hydrops fetalis = Hb Barts = 4 deletions
HbH = 3 allele deletion

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8
Q

defect in beta thal?

A

point mutations in splice sites and promoter sequences

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9
Q

crew cut on skull XR, chipmunk facies?

A

beta thal major - homozygous absence of beta chain
extramedullary hematopoiesis –> HSM
increased risk of parvo–> aplastic crisis
increased HbF expression

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10
Q

what enzymes does lead poisoning inhibit?

A

d-ALA dehydratase, ferrochelatase (first and last steps of heme synthesis)
inhibition of RNA degradation –> basophilic stippling

** increased protoporphyrin

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11
Q

tx of lead poisoning in adults vs kids?

A

adults: dimercaprol, EDTA
kids: succimer

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12
Q

hereditary cause of sideroblastic anemia?

A

d-ALA synthase defect

X-linked

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13
Q

causes of sideroblastic anemia?

A

heritable
myelodysplastic syndromes
alcohol, lead, B6 deficiency, Cu deficiency, INH

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14
Q

findings in sideroblastic anemia?

A

increased iron, increased ferritin
normal/decreased TIBC

iron-laden Prussian blue-stained mitochondria

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15
Q

increased homocysteine, normal MMA?

A

folate deficiency
NO neurologic sx
malnutrition, malabsorption, MTX/trimethoprim/phenytoin

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16
Q

increased homocystein and MMA?

A

B12 deficiency
neurologic sx
vegans, malabsorption, pernicious anemia, Diphyllobothrium, gastrectomy

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17
Q

FTT, developmental delay, megaloblastic anemia refractory to folate/B12 tx?

A

orotic aciduria - AR
defect in UMP synthase –> can’t convert orotic acid to UMP
tx: UMP (bypass defective enzyme)

** NO hyperammonemia (OTC deficiency has hyperammonemia)

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18
Q

findings of intravascular vs extravascular hemolysis?

A

intravascular: decreased haptoglobin, increased LDH, schistos, retics; Hb, hemosiderin, urobilinogen in urine
extravascular: spherocytes, increased LDH, unconjugated hyperbili; nothing in urine

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19
Q

mechanism of anemia of chronic dz?

A

inflammation –> increased hepcidin release from liver
increased binding of ferroportin –> decreased iron transport
decreased release of iron from macrophages –> anemia

** decreased iron, decreased TIBC, increased ferritin

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20
Q

HbC vs HbS?

A

HbC: glu –> lys
HbS: glu –> val

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21
Q

pyruvate kinase deficiency –> anemia?

A

AR

decreased ATP –> rigid RBCs

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22
Q

Coombs - hemolytic anemia, pancytopenia, venous thrombosis?

A

PNH
impaired synthesis of GPI anchor (protects RBCs from complement)
acquired from hematopoietic stem cell
tx: eculizumab = complement inhibitor

    • CD55/59 negative RBCs
    • increased incidence of ALL/AML
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23
Q

things associated with warm AIHA?

A

SLE
CLL
alpha methyldopa

** IgG

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24
Q

things associated with cold AIHA?

A

CLL
Mycoplasma
mono

** IgM

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25
steroid effects on CBC?
neutrophilia: decrease adhesion molecule activation lymphopenia: causes lymphocyte apoptosis eosinopenia: sequesters eos in LNs
26
microcytic anemia, GI and kidney disease, neurologic/mental sx?
lead poisoning ferrochelatase and d-ALA dehydratase inhibited accumulation of protoporphyrin and d-ALA
27
painful abdomen, port wine urine, polyneuropathy, psych disturbances?
AIP defective porphobilinogen deaminase accumulation of porphobilinogen, d-ALA, coporphobilinogen tx: glucose and heme (inhibit ALA synthase)
28
blistering cutaneous photosensitivity, tea colored urine?
porphyria cutanea tarda defective uroporphyrinogen decarboxylase accumulation of uroporphyrin
29
nausea, vom, gastric bleeding, lethargy, scarring --> GI obstruction?
iron poisoning --> cell death via membrane lipid peroxidation tx: deferoxamine or deferasirox chelation, dialysis
30
factors included in PT?
``` I II V VII X ```
31
factors included in PTT?
all except VII and XIII
32
hemarthroses, easy bruising/bleeding after trauma/surg?
hemophilias (a = VIII, b = IX, c = XI) (c = AR, a and b are XLR) increased PTT, PT normal tx: desmopressin + factor concentrate
33
Bernard Soulier?
GpIb defect --> platelet-vWF adhesion defect large platelets ** no agglutination on ristocetin cofactor assay
34
Glanzmann?
GpIIb/IIIa defect --> platelet-platelet aggregation defect no platelet clumping ** agglutination with ristocetin cofactor assay
35
anti-GPIIa/IIIb antibodies?
ITP splenic macrophages consume platelet-Ab complexes megakaryocytes on biopsy tx: steroids, IVIG
36
neurologic and renal sx, fever, TCP, MAHA?
TTP - inhib/deficiency of ADAMTS13 decreased degradation of vWF multimers --> increased platelet adhesion --> increased aggregation and thrombosis schistos and increased LDH ** tx: plasmapheresis, steroids
37
mild bleeding d/o, increased bleed time and PT, normal PT?
vWD - AD decreased carrying of FVIII --> increased PTT decreased platelet-vWF adhesion --> increased bleed time * * decreased aggultination with ristocetin cofactor assay * * tx: desmopressin
38
minimal increase in PTT after heparin administration?
antithrombin deficiency | inherited or acquired (nephrotic syndrome/renal failure)
39
MCC of inherited hypercoagulability in whites?
factor V Leiden | mutant factor V, resistant to degradation by protein C
40
prothrombin gene mutation?
3' UTR mutation --> increased production --> increased plasma levels, venous clots
41
what's in cryoprecipitate?
``` fibrinogen FVIII FXIII vWF fibronectin ```
42
what is a leukemoid rxn?
acute inflammatory response to infx increased WBC with left shift increased leukocyte alkaline phosphatase (decreased in CML)
43
Hodgkin vs Non Hodgkin? (aka good Hodgkin vs bad Hodgkin)
- Hodgkin: localized, Reed-Sternberg, bimodal, EBV, constitutional sx - NHL: multiple nodes, B cells, peak 20-40yo, HIV/autoimmune, fewer constitutional sx
44
markers for Reed Sternberg cell?
CD15 CD30 ``` |\_____/| |[o] [o]| | V | | | -ooo---ooo- ```
45
good prognosis forms of Hodgkin?
strong stromal or lymphocytic rxn against RS cells | lymphocyte rich nodular sclerosing forms
46
starry sky, sheets of lymphocytes with interspersed macrophages?
``` EBV t(8;14) - c-myc and heavy IgG EBV assoc Africa endemic form: jaw lesion sporadic: pelvis, abdomen ```
47
MC NHL in adults?
diffuse large B cell lymphoma | 20% children
48
painless waxing and waning lymphadenopathy; nodular small cleaved cells?
follicular lymphoma t(14;18) - bcl-2 and heavy IgG indolent course
49
CD5+ lymphoma, older males?
mantle cell lymphoma | t(11;14) - cyclin D1 and heavy IgG
50
adults with cutaneous lesions, lytic bone lesions, hyperCa; Japan/West Af/Caribbean?
adult T-cell lymphoma | caused by HTLV
51
skin patches/plaques, atypical CD4+ cells with "cerebriform" nuclei?
mycosis fungoides = cutaneous T cell lymphoma | may progess to Sezary syndrome = T cell leukemia
52
fried egg plasma cells, large amts of IgG and IgA?
MM CRAB: hyperCa, renal, anemia, bone lesions (lytic)/back pain assoc with primary amyloidosis ** M spike, Bence Jones protein, rouleaux, Ig inclusions in plasma cells
53
hyperviscosity, M spike of IgM?
Waldenstrom Macroglobulinemia
54
asymptomatic monoclonal plasma cell expansion?
MGUS | develop MM at 1-2% per year
55
neutrophils with bilobed nuclei?
Pseudo-Pelger-Huet anomaly | seen after chemo
56
children < 15, Down syndrome assoc, TdT+ ?
ALL T-cell: mediastinal mass pre-Bcell: CD10+ may spread to CNS and testes ** t(12;21) has better prognosis
57
adults > 60, AIHA, smudge cells?
CLL/SLL CD20+, CD5+ (CLL has increased peripheral blood lymphocytosis vs SLL)
58
adults, dry BM tap, filamentous cell projections?
hairy cell leukemia TRAP + staining tx: cladribine, pentostatin
59
60-70 yo, auer rods?
AML assoc with Down syndrome t(15;17) = M3 - peroxidase + cytoplasmic inclusions, responds to ATRA ** DIC = common presentation
60
45-85 yo, splenomegaly, metamyelocytes and neutrophils?
CML t(9;22) = BCR-ABL blast crises VERY low LAP tx: imatinib
61
child with lytic bone lesions and skin rash; or recurrent otitis media with mastoid mass?
Langerhans cell histiocytosis do not stimulate T cells with Ag presentation S100 + (mesodermal origin) and CD1a + ** Birbeck granules = tennis racket/rod shaped, seen on EM
62
JAK2 mutation+ myeloproliferative disorders?
polycythemia vera essential thrombocytosis myelofibrosis
63
intense itching after hot shoer; sever burning pain and red-blue coloration?
increased hematocrit, assoc with JAK2 mutation | can get episodic blood clots of extremities
64
essential thrombocytosis?
JAK2 overproduction of abnormal platelets - bleeding, thrombosis enlarged megakaryocytes in BM
65
teardrop RBCs, immature myeloid cells, dry tap, splenomegaly?
myelofibrosis | BM obliteration 2/2 increased fibroblast activity in response to monoclonal proliferation