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step 1 - path > hem/onc > Flashcards

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
Q

steroid effects on CBC?

A

neutrophilia: decrease adhesion molecule activation
lymphopenia: causes lymphocyte apoptosis
eosinopenia: sequesters eos in LNs

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26
Q

microcytic anemia, GI and kidney disease, neurologic/mental sx?

A

lead poisoning
ferrochelatase and d-ALA dehydratase inhibited
accumulation of protoporphyrin and d-ALA

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27
Q

painful abdomen, port wine urine, polyneuropathy, psych disturbances?

A

AIP
defective porphobilinogen deaminase
accumulation of porphobilinogen, d-ALA, coporphobilinogen

tx: glucose and heme (inhibit ALA synthase)

28
Q

blistering cutaneous photosensitivity, tea colored urine?

A

porphyria cutanea tarda
defective uroporphyrinogen decarboxylase
accumulation of uroporphyrin

29
Q

nausea, vom, gastric bleeding, lethargy, scarring –> GI obstruction?

A

iron poisoning –> cell death via membrane lipid peroxidation

tx: deferoxamine or deferasirox chelation, dialysis

30
Q

factors included in PT?

A 
I
II
V
VII
X
31
Q

factors included in PTT?

A

all except VII and XIII

32
Q

hemarthroses, easy bruising/bleeding after trauma/surg?

A

hemophilias
(a = VIII, b = IX, c = XI)
(c = AR, a and b are XLR)
increased PTT, PT normal

tx: desmopressin + factor concentrate

33
Q

Bernard Soulier?

A

GpIb defect –> platelet-vWF adhesion defect
large platelets

** no agglutination on ristocetin cofactor assay

34
Q

Glanzmann?

A

GpIIb/IIIa defect –> platelet-platelet aggregation defect
no platelet clumping

** agglutination with ristocetin cofactor assay

35
Q

anti-GPIIa/IIIb antibodies?

A

ITP
splenic macrophages consume platelet-Ab complexes
megakaryocytes on biopsy
tx: steroids, IVIG

36
Q

neurologic and renal sx, fever, TCP, MAHA?

A

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
Q

mild bleeding d/o, increased bleed time and PT, normal PT?

A

vWD - AD
decreased carrying of FVIII –> increased PTT
decreased platelet-vWF adhesion –> increased bleed time

    • decreased aggultination with ristocetin cofactor assay
    • tx: desmopressin
38
Q

minimal increase in PTT after heparin administration?

A

antithrombin deficiency

inherited or acquired (nephrotic syndrome/renal failure)

39
Q

MCC of inherited hypercoagulability in whites?

A

factor V Leiden

mutant factor V, resistant to degradation by protein C

40
Q

prothrombin gene mutation?

A

3’ UTR mutation –> increased production –> increased plasma levels, venous clots

41
Q

what’s in cryoprecipitate?

A 
fibrinogen
FVIII
FXIII
vWF
fibronectin
42
Q

what is a leukemoid rxn?

A

acute inflammatory response to infx
increased WBC with left shift
increased leukocyte alkaline phosphatase (decreased in CML)

43
Q

Hodgkin vs Non Hodgkin? (aka good Hodgkin vs bad Hodgkin)

A
  • Hodgkin: localized, Reed-Sternberg, bimodal, EBV, constitutional sx
  • NHL: multiple nodes, B cells, peak 20-40yo, HIV/autoimmune, fewer constitutional sx
44
Q

markers for Reed Sternberg cell?

A

CD15
CD30

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45
Q

good prognosis forms of Hodgkin?

A

strong stromal or lymphocytic rxn against RS cells

lymphocyte rich nodular sclerosing forms

46
Q

starry sky, sheets of lymphocytes with interspersed macrophages?

A 
EBV
t(8;14) - c-myc and heavy IgG
EBV assoc
Africa endemic form: jaw lesion
sporadic: pelvis, abdomen
47
Q

MC NHL in adults?

A

diffuse large B cell lymphoma

20% children

48
Q

painless waxing and waning lymphadenopathy; nodular small cleaved cells?

A

follicular lymphoma
t(14;18) - bcl-2 and heavy IgG
indolent course

49
Q

CD5+ lymphoma, older males?

A

mantle cell lymphoma

t(11;14) - cyclin D1 and heavy IgG

50
Q

adults with cutaneous lesions, lytic bone lesions, hyperCa; Japan/West Af/Caribbean?

A

adult T-cell lymphoma

caused by HTLV

51
Q

skin patches/plaques, atypical CD4+ cells with “cerebriform” nuclei?

A

mycosis fungoides = cutaneous T cell lymphoma

may progess to Sezary syndrome = T cell leukemia

52
Q

fried egg plasma cells, large amts of IgG and IgA?

A

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
Q

hyperviscosity, M spike of IgM?

A

Waldenstrom Macroglobulinemia

54
Q

asymptomatic monoclonal plasma cell expansion?

A

MGUS

develop MM at 1-2% per year

55
Q

neutrophils with bilobed nuclei?

A

Pseudo-Pelger-Huet anomaly

seen after chemo

56
Q

children < 15, Down syndrome assoc, TdT+ ?

A

ALL
T-cell: mediastinal mass
pre-Bcell: CD10+
may spread to CNS and testes

** t(12;21) has better prognosis

57
Q

adults > 60, AIHA, smudge cells?

A

CLL/SLL
CD20+, CD5+
(CLL has increased peripheral blood lymphocytosis vs SLL)

58
Q

adults, dry BM tap, filamentous cell projections?

A

hairy cell leukemia
TRAP + staining
tx: cladribine, pentostatin

59
Q

60-70 yo, auer rods?

A

AML
assoc with Down syndrome
t(15;17) = M3 - peroxidase + cytoplasmic inclusions, responds to ATRA

** DIC = common presentation

60
Q

45-85 yo, splenomegaly, metamyelocytes and neutrophils?

A

CML
t(9;22) = BCR-ABL
blast crises
VERY low LAP

tx: imatinib

61
Q

child with lytic bone lesions and skin rash; or recurrent otitis media with mastoid mass?

A

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
Q

JAK2 mutation+ myeloproliferative disorders?

A

polycythemia vera
essential thrombocytosis
myelofibrosis

63
Q

intense itching after hot shoer; sever burning pain and red-blue coloration?

A

increased hematocrit, assoc with JAK2 mutation

can get episodic blood clots of extremities

64
Q

essential thrombocytosis?

A

JAK2
overproduction of abnormal platelets - bleeding, thrombosis
enlarged megakaryocytes in BM

65
Q

teardrop RBCs, immature myeloid cells, dry tap, splenomegaly?

A

myelofibrosis

BM obliteration 2/2 increased fibroblast activity in response to monoclonal proliferation

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