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COMLEX Level 1 > Hematology > Flashcards

Hematology Flashcards

1
Q

α-thalassemia

A

minima: αα/α- (silent)
Minor: α-/α- or αα/– (mild)
Hemoglobin H disease: α-/– (moderate)

microcytic hypochromic anemia
Asian → cis
African → trans

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2
Q

ß-thalassemia

A

microcytic, hypochromic anemia
↑ HbA2
Minor → heterozygote
Major → homozygote

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3
Q

Sickle cell

A

Glutamic acid → Valine on the ß-chain

low O2 and acidosis trigger sickling

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4
Q

HbC disease

A

Glutamic Acid → Lysine on the ß chain

target cells

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5
Q

Ferritin

A

primary storage form of iron

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6
Q

Acute Intermittent prophyria

A 
defective porphobilinogen deaminase
↑ porphobilinogen
Painful abdomen
port wine-colored urine
polyneuropathy
psychological 
alcohol, starvation
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7
Q

Porphyria cutanea tarda

A 
defective uroporphyrinogen decarboxylase
↑ Uroporphyrin
Blistering skin photosensitivity
hyperpigmentation
associated with Hep C
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8
Q

↑ PTT

A

intrinsic pathway defect

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9
Q

↑ PT

A

Extrinsic pathway defect

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10
Q

Hemophilia ABC

A

A → factor VIII (8)
B → factor IX (9)
C → factor XI (11)

Hemarthroses
easy bruising
intrinsic pathway defects

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11
Q

Bernard-Soulier

A

GpIb

platelet-vWF adhesion defect

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12
Q

Glanzmann thrombasthenia

A

GpIIb/IIIa

platelet-platelet adhesion defect

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13
Q

TTP and HUS

A

Thombocytopenia + hemolyic anemia + AKI

HUS → hx of bloody diarrhea (commonly EHEC)
TTP → triad + fever + neuro symptoms
→ ADAMTS13

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14
Q

von Willebrand

A

↓vWF
platelet-vWF adhesion defect
↑ PTT

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15
Q

DIC

A

disseminated intravascular coagulation
widespread activation of clotting leads to ↓ clotting factors →
bleeding state

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16
Q

Factor V leiden

A

hypercoagulability

DNA point mutation of guanine → adenine

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17
Q

Hodgkin lymphoma

A

localized, single group of nodes
Reed-Sternberg cells → owl eyes, CD15, CD30
B-cell origin
EBV

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18
Q

Burkitt lymphoma

A 
Non-Hodgkin lymphoma
t(8;14) (c-myc; heavy chain Ig) (Burk8)
starry sky
Jaw lesions in endemic African version
Pelvis or abdomen in sporadic form
EBV
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19
Q

Diffuse large B-cell lymphoma

A

BCL-2, BCL-6

common in adults

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20
Q

Follicular lymphoma

A

t(14;18) (heavy chain Ig ; BCL-2)

painless waxing and waning lymphadenopathy

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21
Q

Mantle cell lymphoma

A

t(11;14) (cyclin D1 ; heavy chain Ig)
Males (MANtle)
agressive

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22
Q

Marginal zone

A 
t(11;18) (cyclin D1 ; BCL-2)
chronic inflammation (Sjogrens, chronic gastritis)
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23
Q

Primary central nervous system lymphoma

A

AIDs defining illness
Confusion, memory loss, seizures
ring enhancing brain lesion

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24
Q

Adult T-cell lymphoma

A

HTLV (IV drug abuse)
Japan, Africa, Caribbean
lytic bone lesions, hypercalcemia

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25
Q

Mycosis fungoides/Sezary syndrome

A

skin patches and plaques
atypical CD4 cells with cerebriform nuclei
Pautrier microabcesses (cancer cell aggregates in epidermis)

26
Q

ALL (acute lympoblastic leukemia)

A 
Children
asx with Down syndrome
TdT+ 
CD10+
mediastinal mass
t(12;21) → better prognosis
27
Q 
CLL (chronic lymphycytic leukemia)
aka SLL (small lymphocystic lymphoma)
A

Age 60+
CD5+
CD20+
CD23+
smudge cells in blood smear (CLL = crushed little lymphocytes)
Richter transformation → CLL/SLL into aggressive lymphoma, usually diffuse large B-cell

28
Q

Hairy cell leukemia

A

tumor cells have hair like projections
marrow fibrosis
stains TRAP+
BRAF mutation

29
Q

AML (acute myelogenous leukemia)

A

Auer rods: myeloperoxidase + cytoplasmic inclusions
↑↑↑ circulating myeloblasts
age 65

Risk factors:
Down syndrome
exposure to radiation, or chemotherapy
t(15;17) (APL form of AML, asx with Auer rods, responds to all-trans retinoic acid)

30
Q

CML (Chronic myelogenous leukemia)

A

age 45-85
t(9;22), BCR-ABL → Philadelphia chromosome
↓ LAP (leukocyte alkaline phosphatase)
EML4-ALK

31
Q

Langerhans cell histiocytosis

A

lytic bone lesions + skin rash
recurrent otitis media with mastoid bone mass
Birbeck granules → tennis rackets on EM
S-100

32
Q

Warfarin

A

inhibits epoxide reductase in Vit K
↓ factors 2, 7, 9, 10, protein S, protein C
Initially hypercoaguable

33
Q

Heparin

A

↑ antithrombin → ↓action of IIa (thrombin) and Xa

Low molecular weight versions are:
enoxaparin, dalteparin → mostly Xa
fondaparinux → only Xa

34
Q

Bivalirudin

A

direct thrombin inhibitor

used when HIT occurs

35
Q

Argatroban

A

direct thrombin inhibitor

used when HIT occurs

36
Q

Dabigatran

A

direct thrombin inhibitor

used when HIT occurs

37
Q

Apixaban

A

Direct factor Xa inhibitor

38
Q

Rivaroxaban

A

Direct factor Xa inhibitor

39
Q

ADP receptor inhibitors

A 
Clopidogrel, ticlopidine, ticagrelor, prasugrel
block ADP (P2Y12) receptor → ↓expression of glycoproteins IIb/IIIa
40
Q

Clopidogrel

A

ADP receptor inhibitors

41
Q

ticlopidine

A

ADP receptor inhibitors

42
Q

ticagrelor

A

ADP receptor inhibitors

43
Q

prasugrel

A

ADP receptor inhibitors

44
Q

Glycoprotein IIb/IIIa inhibitors

A

Abciximab, eptifibatide, tirofan

bind to IIa/IIIb on platelets, inhibiting aggregation

45
Q

Abciximab

A

Glycoprotein IIb/IIIa inhibitors

46
Q

eptifibatide

A

Glycoprotein IIb/IIIa inhibitors

47
Q

tirofiban

A

Glycoprotein IIb/IIIa inhibitors

48
Q

Multiple myeloma

A 
CRAB
hyperCalcemia
Renal involvement
Anemia
Bone lytic lesions (punched out lesion)

M spike
↑ Ig → Ig in urine (Bence Jones protein)
Rouleaux formation (RBC stacks)
clock face plasma cells

49
Q

CD3

50
Q

CD28

51
Q

CD19, 20, 21

52
Q

CD14

A

macrophage

53
Q

CD16

54
Q

CD56

55
Q

IL-2

56
Q

IL-3

A

bone marrow stem cells

57
Q

IL-4

A

IgE class switching
Tcells → Th2 cells
B cell growth

58
Q

IL-5

A

IgA class switching

59
Q

CYP-450 inducers

A 
Phenytoin
Phenobarbital
Carbamezapine
Rifampin
St. John's Wort

↑ drug clearance

60
Q

CYP-450 inhibitors

A 
Antibiotics (metronidazole, macrolides)
Azole Antifungals
Amiodarone
Cimetidine
Grapefruit juice

↑ drug concentration → overdose

COMLEX Level 1 (13 decks)

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