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EBSE > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Poikilocytosis

A

varying shapes of RBCs

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2
Q

dense vs alpha granules in platelets

A

dense - ADP, Ca

alpha - vWF, fibrinogen

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3
Q

vWF receptor

A

Gp1b

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4
Q

fibrinogen receptor

A

Gp2b/3a

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5
Q

hypersegmented PMNs seen in what condition

A

b12/folate deficiency

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6
Q

cell surface marker for macrophages

A

CD14

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7
Q

Hemophilia A and B - what factors are deficient?

A

A - 8

B - 9

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8
Q

vit K dependent clotting factors

A

2,7,9,10, protein C and S

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9
Q

action of proteins C and S

A

activated protein C cleaves and inactivates 5a and 8a

protein S is a cofactor for C

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10
Q

2 conditions in which acanthocytes are present

A

liver disease

abetalipoproteinemia

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11
Q

causes of basophilic stipling of RBCs (3)

A

thalassemias, anemia of chronic dz, lead poisoning

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12
Q

bite cells

A

G6PD def

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13
Q

pathologic cell in sideroblastic anemia

A

RINGED sideroblast

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14
Q

target cells seen in (4)

A

HbC dz, asplenia, liver dz, thalassemia

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15
Q

Heinz bodies

A

oxidized iron > denatured hgb precipitates and damages membrane
seen in G6PD def, and heinz-like inclusions seen in alpha thalassemia

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16
Q

Howell Jolly bodies

A

basophilic inclusions of nuclear remnants

seen in asplenia or functional hyposplenia or w/ naphthalene ingestion

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17
Q

iron panel in iron def anemia

A

dec iron, inc TIBC, dec ferritin

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18
Q

Plummer Vinson syndrome

A

iron def anemia, esophageal webs, atrophic glossitis

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19
Q

of gene deletions and presentation in alpha thalassemia

A

1-2 deletion - subclinical anemia
3 deletions - HbH dz, excess beta globin forms beta tetramers (HbH)
4 deletions - excess gamma globin > HbBarts > hydrops fetalis > stillbirth

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20
Q

beta thalassemia major sx

A

severe anemia requiring transfusions (possibility for 2ndary hemochromatosis)
marrow expansion > skeletal deformities
chipmunk facies
increased HbF

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21
Q

HbS/Beta thalassemia heterozygotes - severity of disease

A

mild to moderate sickle cell depending on amt of beta production

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22
Q

lead poisoning - type of anemia caused? signs/sx?

A 
sideroblastic due to dec heme production
LEAD:
lead lines on gingivae (Burton's lines)
encephalopathy / erythrocyte basophilic stipling
abdominal colic / anemia
drops - wrist and foot drop
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23
Q

tx for lead poisoning

A

dimercaprol and EDTA, succimer for kids

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24
Q

causes of sideroblastic anemia - 1 hereditary, 3 reversible

A

hereditary - XL defect in delta-ALA synthase

reversible - ETOH, lead, INH

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25
Q

iron panel in sideroblastic anemia

A

inc iron, normal TIBC, inc ferritin

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26
Q

tx of sideroblastic anemia

A

pyridoxine (B6)

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27
Q

labs for megaloblastic anemia to distinguish folate from B12 def

A

folate - inc homocysteine, nl methylmalonic acid (MMA)

B12 - inc homocysteine and MMA

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28
Q

neuro sx of b12 def

A 
subacute combined degeneration:
peripheral neuropathy
posterior columns - vibration / proprioception
lateral CST - spasticity
dementia
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29
Q

lab findings for intravascular vs extravascular hemolysis

A

intra - dec haptoglobin, inc LDH, hemoglobinuria

extra - inc LDH, inc unconj bili

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30
Q

iron panel for anemia of chronic dz

A

dec iron, dec TIBC, inc ferritin

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31
Q

hereditary spherocytosis is generally associated with mutation in ___, which needs to interact with ____ & _____ to make the correct shape

A

ankyrin, spectrin, band 3

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32
Q

mutations in ____ will result in acanthocytic hereditary spherocytosis, while mutations in ____ result in pincered HS

A

beta spectrin

band 3

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33
Q

hereditary spherocytosis and elliptocytosis - defect in vertical or horizontal interactions?

A

spherocytosis - vertical

elliptocytosis - horizontal

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34
Q

G6PD def - inheritance? PBS signs?

A

XL

Heinz bodies and bite cells

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35
Q

pyruvate kinase def - inheritance? pathogenesis? findings?

A

AR
defect in pyruvate kinase > dec ATP > rigid RBCs
hemolytic anemia in newborn

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36
Q

paroxysmal nocturnal hemoglobinuria - cause

A

inc complement mediated RBC lysis due to acquired mutation in HSC

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37
Q

paroxysmal nocturnal hemoglobinuria - triad of findings

A

hemolytic anemia, pancytopenia, venous thrombosis

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38
Q

tx of PNH

A

eculizumab

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39
Q

what precipitates sickling in sickle cell?

A

low O2 or dehydration

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40
Q

complications of sickle cell (5)

A

aplastic crises w/ infxn (parvo B19 esp)
autosplenectomy / splenic sequestration crisis
salmonella osteomyelitis
pain crises - dactylitis, acute chest syndrome, avascular necrosis
renal papillary necrosis and microhematuria

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41
Q

enzymes inhibited by lead poisoning

A

ferrochelatase and ALA dehydratase

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42
Q

acute intermittent porphyria - affected enzyme

A

porphobilinogen deaminase

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43
Q

acute intermittent porphyria - sx and tx

A 
5 Ps:
painful abd
port wine colored urine
polyneuropathy
psych disturbanes
precipitated by drugs

tx - glucose and heme

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44
Q

MC type of porphyria

A

porphyria cutanea tarda

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45
Q

porphyria cutanea tarda - affected enzyme and sx

A

uroporphyrinogen decarboxylase

blistering cutaneous photosensitivity

46
Q

Bernard Soulier syndrome

A

dec Gp1b > defect in platelet to vWF adhesion

47
Q

Glanzmann’s thrombasthenia

A

dec Gp2b/3a > defect in platelet aggregation

48
Q

ITP - pathogenesis

A

anti-Gp2b/3a antibodies > splenic macrophages eat platelet/ab complexes > less thrombocytopenia

49
Q

TTP - pathogenesis

A

def of ADAMSTS 13 (vWF metalloprotease) > dec degradation of vWF multimers > inc platelet aggregation > dec platelet survival

50
Q

TTP - pentad of sx

A 
neuro sx
renal sx
fever
thombocytopenia
microangiopathic hemolytic anemia
51
Q

MC hereditary bleeding disorder

A

von willebrand’s

52
Q

von willebrand dz inheritance

A

AD

53
Q

tx for von willebrand dz

A

DDAVP - releases vWF stored in endothelium

54
Q

causes of DIC

A

Sepsis, Trauma, Obstetric complications, acute Pancreatitis, Malignancy, Nephrotic syndrome, Transfusion
STOP Making New Thrombi

55
Q

factor V leiden

A

V that is resistant to degradation by activated protein C > MC cause of inherited hypercoagulability in whites

56
Q

what is cryoprecipitate useful for

A

factor deficiencies involving fibrinogen and factor 8

57
Q

hodgkin’s vs NHL - location/spread? age? which is more assoc w/ EBV? constitutional sx?

A

hodgkin - localized w/ contiguous spread
2 peaks - young adult and >55
assoc w/ EBV
B sx common (fever, night sweats, wt loss)

NHL - multiple sites w/ noncontiguous spread
20-40 yo
fewer constitutional sx

58
Q

main cell of hodgkin’s

A

Reed Sternberg cell

59
Q

form of hodgkin’s w/ best prognosis

A

nodular sclerosing (also most common)

60
Q

“starry sky” appearance

A

burkitt’s

61
Q

burkitt’s translocation and gene

A

t(8;14), c-myc

62
Q

MC adult NHL

A

diffuse large B cell lymphoma

63
Q

mantle cell lymphoma translocation

A

t(11;14)

64
Q

follicular lymphoma translocation and gene

A

t(14;18), bcl-2

65
Q

mycosis fungiodes / sezary syndrome

A

T cell NHL w/ cutaneous manifestations

66
Q

MC primary tumor arising from bone in elderly

A

multiple myeloma

67
Q

Waldenstrom’s macroglobulinemia

A

M spike of IgM, hyperviscosity sx but no bone lesions

68
Q

findings in multiple myeloma (6)

A 
inc susceptibility to infxn
primary amyloidosis
punched out lytic bone lesions
M spike on protein electrophoresis
Ig light chains in urine - Bence Jones protein
Rouleaux formation
69
Q

sx of multiple myeloma (4)

A 
CRAB - 
hyperCalcemia
Renal insufficiency
Anemia
Bone lytic lesions/Back pain
70
Q

good prognosis tranlsocation for ALL

A

t(12;21)

71
Q

smudge cells

A

CLL

72
Q

hairy cell leukemia - who gets it, special stain

A

adults/elderly

TRAP - tartrate resistant acid phosphatase

73
Q

AML M3 - genetics stuff, presentation/PBS finding, tx

A

Acute promyelocytic leukemia
t(15;17)
auer rods, presents w/ DIC
tx w/ all trans retinoic acid

74
Q

CML - genetics stuff, tx

A

t(9;22) philadelphia chromosome, bcr-abl

tx w/ imatinib

75
Q

Langerhans cell histiocytosis - presentation, microscopic finding

A 
child w/ lytic bone lesions and skin rash
Birbeck granules ("tennis rackets" on EM)
76
Q

gene mutation assoc with several myeloproliferative disorders

A

JAK2

77
Q

heparin mech

A

cofactor in activation of antithrombin, dec thrombin and factor 10a

78
Q

antidote for heparin

A

protamine sulfate

79
Q

coag tests to follow for heparin and warfarin

A

heparin - PTT

warfarin - PT (INR)

80
Q

lower molec weight heparins act more on

A

factor Xa

81
Q

mech for heparin induced thrombocytopenia

A

IgG against heparin bound to platelet factor 4 > activation of platelets > thrombosis and thrombocytopenia

82
Q

alternative to heparin for HIT patients

A

lepirudin, bivalirudin (derived from anticoag found in leeches

83
Q

alteplase is

A

tPA

84
Q

antidote for thrombolytics

A

aminocaproic acid (inhibs fibrinolysis)

85
Q

sx of aspirin overdose

A

resp alkalosis and metab acidosis

86
Q

clopidogrel, ticlopidine, prasgurel, ticagrelor - drug class

A

ADP receptor inhibitors (irreversible)

87
Q

cilostazol, dipyridamole - drug class, use, SEs

A

PDE3 inhib
claudication, coronary vasodilation, prevention of stroke/TIA, angina prophylaxis
nausea, HA, facial flushing, hypotension, abd pain

88
Q

abciximab, eptifibatide, tirofiban - class, use

A

Gp2b/3a inhibitors

acute coronary syndromes, percutaneous transluminal coronary angioplasty

89
Q

methotrexate acts on what enyzme

A

dihydrofolate reductase

90
Q

methotrexate tox

A

**myelosuppression
fatty change in liver
mucositis
teratogen

91
Q

rescue drug for methotrexate induced myelosuppression

A

leucovorin

92
Q

5FU - class, tox

A

antimetabolite

**myelosuppression
photosensitivity

93
Q

cytarabine - class, tox

A

antimetabolite

leukopenia, thrombocytopenia, megaloblastic anemia

94
Q

azathioprine / 6MP - class, tox

A

antimetabolite

**Myelosuppression
GI/liver tox
can’t take w/ allopurinol

95
Q

dactinomycin - class, general use, tox

A

antitumor abx
used for childhood tumors
myelosuppression

96
Q

doxorubicin/adriamycin - class, tox

A

antitumor abx

**cardiotox
myelosuppression, alopecia

97
Q

bleomycin - class, tox

A

antitumor abx

**pulm fibrosis
skin change, some myelosuppression

98
Q

cyclophosphamid/ifosfamide - class, tox

A

alkylating agent

**hemorrhagic cystitis (prevent w/ mesna)

99
Q

nitrosoureas - class, tox

A

alklyating agent

cns tox (dizziness, ataxia)

100
Q

busulfan - class, tox

A

alkylating agent

**pulm fibrosis

101
Q

vinca alkaloids - class, tox

A

MT inhibitor

vincristine- **peripheral neuropathy
vinblastine - blasts bone marrow

102
Q

taxanes - class, tox

A

MT inhibitor

myelosuppression

103
Q

cisplatin/carboplatin - mech, tox

A

cross link DNA

**nephro and acoustic nerve tox

104
Q

etoposide/teniposide - mech, tox

A

inhibit topoisomerase II > DNA degradation

myelosuppression, GI irritation, alopecia

105
Q

hydroxyurea - mech, tox

A

inhibits ribonucleotide reductase > dec DNA synth

myelosuppression, GI upset

106
Q

tamoxifen/raloxifene - mech, tox

A

SERMs (selective estrogen receptor modulators)

tamoxifen - inc risk of endometrial cancer

107
Q

trastuzumab (Herceptin) - mech, tox

A

ab against HER2

**cardiotox

108
Q

imatinib (Gleevec) - mech, tox

A

bcr abl tyrosine kinase inhibitor

fluid retention

109
Q

rituximab - mech, tox

A

anti CD20

NHL, RA

110
Q

vemurafenib - mech, use

A

inhibitor of B-Raf kinase

used in metastatic melanoma

111
Q

bevacizumab - mech, use

A

ab against VEGF

solid tumors

EBSE (18 decks)

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