Hematology Flashcards

(60 cards)

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
Mycosis fungoides/Sezary syndrome
skin patches and plaques atypical CD4 cells with cerebriform nuclei Pautrier microabcesses (cancer cell aggregates in epidermis)
26
ALL (acute lympoblastic leukemia)
``` Children asx with Down syndrome TdT+ CD10+ mediastinal mass t(12;21) → better prognosis ```
27
``` CLL (chronic lymphycytic leukemia) aka SLL (small lymphocystic lymphoma) ```
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
Hairy cell leukemia
tumor cells have hair like projections marrow fibrosis stains TRAP+ BRAF mutation
29
AML (acute myelogenous leukemia)
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
CML (Chronic myelogenous leukemia)
age 45-85 t(9;22), BCR-ABL → Philadelphia chromosome ↓ LAP (leukocyte alkaline phosphatase) EML4-ALK
31
Langerhans cell histiocytosis
lytic bone lesions + skin rash recurrent otitis media with mastoid bone mass Birbeck granules → tennis rackets on EM S-100
32
Warfarin
inhibits epoxide reductase in Vit K ↓ factors 2, 7, 9, 10, protein S, protein C Initially hypercoaguable
33
Heparin
↑ antithrombin → ↓action of IIa (thrombin) and Xa Low molecular weight versions are: enoxaparin, dalteparin → mostly Xa fondaparinux → only Xa
34
Bivalirudin
direct thrombin inhibitor | used when HIT occurs
35
Argatroban
direct thrombin inhibitor | used when HIT occurs
36
Dabigatran
direct thrombin inhibitor | used when HIT occurs
37
Apixaban
Direct factor Xa inhibitor
38
Rivaroxaban
Direct factor Xa inhibitor
39
ADP receptor inhibitors
``` Clopidogrel, ticlopidine, ticagrelor, prasugrel block ADP (P2Y12) receptor → ↓expression of glycoproteins IIb/IIIa ```
40
Clopidogrel
ADP receptor inhibitors
41
ticlopidine
ADP receptor inhibitors
42
ticagrelor
ADP receptor inhibitors
43
prasugrel
ADP receptor inhibitors
44
Glycoprotein IIb/IIIa inhibitors
Abciximab, eptifibatide, tirofan | bind to IIa/IIIb on platelets, inhibiting aggregation
45
Abciximab
Glycoprotein IIb/IIIa inhibitors
46
eptifibatide
Glycoprotein IIb/IIIa inhibitors
47
tirofiban
Glycoprotein IIb/IIIa inhibitors
48
Multiple myeloma
``` 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
CD3
T-cell
50
CD28
T-cell
51
CD19, 20, 21
B-cell
52
CD14
macrophage
53
CD16
NK cell
54
CD56
NK cell
55
IL-2
T cells
56
IL-3
bone marrow stem cells
57
IL-4
IgE class switching Tcells → Th2 cells B cell growth
58
IL-5
IgA class switching
59
CYP-450 inducers
``` Phenytoin Phenobarbital Carbamezapine Rifampin St. John's Wort ``` ↑ drug clearance
60
CYP-450 inhibitors
``` Antibiotics (metronidazole, macrolides) Azole Antifungals Amiodarone Cimetidine Grapefruit juice ``` ↑ drug concentration → overdose