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Step One > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

residual ribosomal RNA

A

bluish color on Wright Giemsa

reticulocytes

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2
Q

contents dense granules

A

in thrombocyte

ADP and Ca

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3
Q

contents alpha granules leukocytes

A

vWF, fibronogen, fibronectin

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4
Q

granules in neutrophil

A

leukocyte alkaline phosphatase
collagenase
lysozyme and lactoferrin

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5
Q

neutrophil chemotactic

A

c5a, IL8, kallikrein, LTB4, platelet activating factor

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6
Q

activation macrophages

A

IFN gamma

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7
Q

initiation of septic shock

A

lipid A from LPS binds CD14 on macs

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8
Q

causes of eosinophilia

A 
neoplasia
asthma
allergic processes
chronic adrenal insufficiency 
parasites
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9
Q

products eosinophils

A

histamine, major basic protein, eosinophil peroxidase, eosinophil cationic protein

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10
Q

basophilia

A

CML

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11
Q

prevention mast cell degranulation

A

cromolyn

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12
Q

costim for T cells

A

CD28

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13
Q

fetal erythropoiesis

A 
yolk sac 3-8 weeks
liver 6-birth
spleen 10-28 weeks
bone marrow 
Young liver synthesizes blood
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14
Q

higher O2 binding fetal Hb

A

less avid binding of 2,3BPG

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15
Q

ABO hemolytic disease

A

O mother with A or B fetus

mild jaundice in neonate within 24 hrs of birth

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16
Q

Hb on electrophoresis

A

A fat santa Claus

A travels furthest

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17
Q

VWF and coag

A

protects and carries factor 8

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18
Q

factor V leiden

A

mutation produces factor V resistant to inhibition by protein C

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19
Q

antithrombin inhibition

A

2,7,9,10,11,12

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20
Q

protein C

A

inactivates 5a and 8a

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21
Q

induction Gp2b/3a

A

ADP binding P2Y12

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22
Q

Ristocetin

A

activates vWF to bind Gp1b

failure occurs in vWF disease and Bernard-Soulier syndrome

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23
Q

causes acanthocyte

A

liver disease, abetalipoproteinemia

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24
Q

burr cell

A

end stage renal disease, liver disease, pyruvate kinase deficiency
more uniform than acanthocyte

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25
Q

causes target cell

A

HbC, asplenia, liver disease, thalassemia

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26
Q

deletions alpha thalassemia

A

cis worse-Asian

trans-African

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27
Q

Hb Barts

A

found in 4 deletions

incompatible with life

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28
Q

HbH

A

found with 3 deletions

excess beta globulin formation B4

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29
Q

cause of beta thalassemia

A

mutations in splice sites and promoter sequences

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30
Q

labs b thal minor

A

increase A2

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31
Q

risk for b thal major

A

B19

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32
Q

labs b thal major

A

HbF

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33
Q

lead inhibition

A

ferrochelatase and ALAD

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34
Q

symptoms lead poisoning

A

lead lines

wrist and foot drop

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35
Q

treatment lead poisoning

A

EDTA and dimercaprol

succimer in children

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36
Q

genetic sideroblastic anemia

A

defect in ALAS

could also be B6 deficit

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37
Q

B12 vs folate

A

increase both lab findings and neuro in B12

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38
Q

tracts B12 def

A

lateral corticospinal tract, spinocerebellar, dorsal column

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39
Q

orotic aciduria

A

inability to convert orotic acid to UMP due to defective UMP synthase
AR

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40
Q

treatment orotic aciduria

A

uridine monophosphate

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41
Q

diff orotic aciduria and ornithine transcarbamylase

A

no hyperammonemia in orotic aciduria

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42
Q

cause Diamond-Blackfan anemia

A

defect in erythroid progenitor cells

increase %HbF

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43
Q

labs intravascular hemolysis

A

decrease haptoglobin

hemoglobinuria, hemosiderinuria and urobilinogen in urine

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44
Q

etiology anemia of chronic disease

A

increase hepcidin inhibits iron transport

give EPO in CKD

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45
Q

cause Fanconi anemia

A

DNA repair defect causing bone marrow failure

short stature, increase tumors/leukemias, cafe-au-lait spots, thumb/radial defects

46
Q

pyruvate kinase deficiency

A

AR
rigid RBCs, increase 23BPG and decreased affinity for O2
leads to hemolytic anemia in newborn

47
Q

cause PNH

A

GPI anchor for DAF

leads to increased leukemias

48
Q

labs PNH

A

CD55/59-

49
Q

complications sickle

A

painful crises, acute chest syndrome, avascular necrosis, renal papillary necrosis and microhematuria (medullary infarcts)

50
Q

smear HbC

A

crystals inside RBCs, target cells

from lysine mutation in beta globin

51
Q

warm agglutination

A

SLE and CLL
IgG mediated
also alpha-methyldopa

52
Q

cold agglutination

A

CLL, mycoplasma, mono

IgM mediated

53
Q

Direct Coombs

A

anti-Ig Ab to patient RBC

54
Q

Indirect Coombs

A

normal RBC to patient serum

55
Q

lab diff iron def and chronic disease anemia

A

decreased transferrin in chronic disease

increase ferritin in chronic disease

56
Q

defective enzyme acute intermittent porphyria

A

porphobilinogen deaminase leads to accumulation of ALA and porphobilinogen
AD

57
Q

symptoms acute intermittent porphyria

A 
painful abdomen
port wine colored urine
polyneuropathy
psychological distubrances
precipitated by drugs-p450 inducers, alcohol, starvation
58
Q

treatment acute intermittent porphyria

A

glucose and heme

59
Q

defective enzyme porphyria cutanea tarda

A

uroporphyrinogen decarboxylase AD

leads to increase uroporphyrin

60
Q

symptoms porphyria cutanea tarda

A

blistering cutaneous photosensitivity and hyperpigmentation

exacerbated by alcohol

61
Q

mechanism iron poisoning

A

cell death due to peroxidation of membrane lipids

62
Q

coagulation studies

A

factor inhibitors will not correct

63
Q

hemophilia C

A

AR

defective 11

64
Q

defect bernard-soulier

A

defective platelet plug formation

decrease Gp1b

65
Q

defect Glanzmann thrombasthenia

A

defect in Gp2b3a

blood smear shows no platelet to platelet clumping

66
Q

labs ITP

A

large megs

Ab to Gp2b3a

67
Q

labs TTP

A

increase LDH
normal coag
increase BT

68
Q

causes DIC

A

sepsis (gram-), trauma, obstetric complications, acute pancreatitis, malignancy, nephrotic syndrome

69
Q

anti-thrombin deficiency

A

diminishes increase in PTT following heparin admin

can be acquired from renal failure-lost in urine leading to decreased inhibition of 2a/10a

70
Q

protein C or S def

A

decreased ability to inactivate 5a/8a

71
Q

protnrhombin mutation

A

in 3’ UTR leads to increased production

leads to venous clots

72
Q

contents cryoprecipitate

A

fibrinogen, factor 8,13, vWF, fibronectin

give for factor deficiencies

73
Q

blood transfusion risks

A

iron overload
hypocalcemia (due to citrate binding Ca)
hyperkalemia

74
Q

associations non-hodgkin lymphoma

A

HIV and autoimmune

75
Q

cell markers hodgkin lymphoma

A

15, 30

76
Q

genetics diffuse large B cell lymphoma

A

bcl2, bcl6

77
Q

presentation follicular lymphoma

A

waxing and waning

78
Q

genetics and association marginal zone lymphoma

A

11;18

associated with chronic inflammation-Sjogren and chronic gastritis

79
Q

primary central nervous system lymphoma

A

associated with HIV/AIDS
pathogenesis involves EBV
needs to be distinguished from toxo

80
Q

ault T cell lymphoma

A

caused by HTLV (IV drug association)

cutaneous lesions, lytic bone lesions and hypercalcemia

81
Q

M spike

A

Waldenstrom macroglobulinemia

82
Q

Pseudo-Pegler-Huet anomaly

A

neutrophils with bilobed nuclei

seen after chemo

83
Q

ALL

A

children, Down syndrome
TdT and CD10+
11;21

84
Q

spread ALL

A

CNS and testes

85
Q

CLL

A

20,23,5+

smudge cells

86
Q

Richter transformation

A

CLL into aggressive lymphoma-diffuse large B cell lymphoma

87
Q

Hairy cell leukemia

A

mature B cell tumor
marrow fibrosis, massive splenomegaly
stains TRAP

88
Q

treatment hairy cell leukemia

A

cladribine, pentostatin

89
Q

transformation CML

A 
to AML or ALL
low LAP (diff from leukemoid reaction)
90
Q

itching after hot shower

A

polycythemia vera

decrease EPO

91
Q

presentation essential thrombocythemia

A

massive megs and platelets, symptoms of bleeding and thrombosis

92
Q

cause relative polycythemia

A

decrease plasma volume from dehydration and burns

93
Q

presentation langerhans cell histiocytosis

A

lytic bone lesions and skin rash

recurrent otitis media

94
Q

cell markers langerhans cell histiocytes

A

S100 (mesodermal) and CD1a

95
Q

HIT

A

IgG ab against heparin platelet factor 4

96
Q

reverse dabigatran

A

idarucizumab

97
Q

adverse cytarabine

A

pancytopenia

98
Q

MOA 5FU

A

bioactivated by 5-FdUMP and complexes with thymidylate synthase and folic acid
inhibits thymidylate synthase

99
Q

leucovorin

A

increases effects 5FU

100
Q

busulfan vs bleomycin

A

minimal BM suppression with bleo

101
Q

MOA cyclophosphamide

A

crosslink at guanine N7

requires activation by liver

102
Q

MOA mesna

A

binds toxic metabolites

103
Q

bevacizumab

A

inhibits VEGF

104
Q

erlotinib

A

inhibits EGFR tyrosine kinase for non-small cell lung cancer

105
Q

cetuximab

A

ab against EGFR
used for colorectal, head and neck cancer
can elevated LFTs

106
Q

adverse rituximab

A

increase risk of PML

107
Q

bortezomib, carfilzomib

A

protease inhibitors
arrest G2-M
can be used for MM and mantle cell lymphoma
can reactivate herpes, cause peripheral neuropathy

108
Q

raloxifene

A

no risk of endometrial carcinoma

109
Q

vemurafenib

A

inhibits BRAF in V600E melanoma

110
Q

rasburicase

A

recombinant uricase that catalyzes uric acid to allantoin

prevention and treatment of tumor lysis syndrome

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