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IBI Test 4 > B3.066 - CBCL Thrombocytopenia > Flashcards

B3.066 - CBCL Thrombocytopenia Flashcards

1
Q

What is TTP

A

pentad characterization 1. fever 2. thrombocytopenia 3. transient neuro deficits 4. renal failure 5. microangiopathic hemolytic anemia

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2
Q

what causes TTP

A

deficiency in ADAMTS13, which cleaves/degrades very high molecular weight vWF, leading to more clotting

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3
Q

who is at higher risk for TTP

A

african american women and pregnant women

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4
Q

what genetic tests can you do fro TTP

A

mutations in ADAMTS13

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5
Q

common causes of TTP

A

ADAMTS13 mutations (Upshaw-Schulman syndrome) HIV Autoimmune disorders preglancy some drugs

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6
Q

symptoms of TTP

A

malaise bleeding altered mental status low grade fever petechiae pallor jaundice confusion seizures

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7
Q

tests for TTP

A

ADAMTS13 activity and circulating ADAMTS13 antibody negative coombs polychromasia elevated LDH elevated bilirubin bc of heme metabolism elevated BUN with kidney failure

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8
Q

differential for TTP

A

sepsis vasculitis

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9
Q

how do you differentiate sepsis from TTP

A

sepsis will have normal ADAMTS13 levels

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10
Q

how do you differentiate vasculitis from TTP

A

not usually associated with microthrombi or hemolytic anemia

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11
Q

how do you differentiate TTP from malignant hypertension

A

retinopathy grade III or IV seen in malignant HT not TTP

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12
Q

how do you differentiate TTP from Autoimmune hemolytic anemia

A

positive coombs test spherocytes rather than schistocytes on PBS, no MAHA

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13
Q

first line of treatment for TTP

A

plasma exchange

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14
Q

what type of plasma is given for TTP and what line of therapy is it and what do you have to be careful of

A

fresh frozen plasma 2nd line, careful not to overload patient so you have to give slowly

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15
Q

are antiplatelets helpful in TTP

A

no

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16
Q

is a platelet transfusion helpful in TTP?

A

no

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17
Q

what is aHUS

A

characterized by microangiopathic hemolytic anemia and thrombocytopenia, prominent in renal insufficiency

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18
Q

how does aHUS happen

A

associated with defects in complement factor H, membrane cofactor CD46 or factor I. Deficiency of complement regulatory proteins leads to activation of alternative complement system

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19
Q

what proteins are associated with activation of alternative complement system in aHUS

A

factor H factor I membrane cofactor protein thrombomodulin

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20
Q

what factors are associated with aberrant activation of alternative complement pathway

A

factor B Factor C3

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21
Q

secondary aHUS

A

initiated by coexisting disease or condition

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22
Q

triggering events that can cause aHUS

A

strep pneumo HIV malignancies pregnancy rheumatologic disease drugs like cyclosporine and tac stem cell transplantation

23
Q

presentation of aHUS

A

microangiopathic hemolytic anemia thrombocytopenia renal failure neurological manifestations are less common no abdominal pain and diarrhea

24
Q

lab tests for aHUS

A

*BUN markedly elevated *Tests for complement *negative culture *ADAMSTS13>10% CBC Peripheral smear (schistocytes) elevated LDH elevated bilirubin negative coombs normal PT, PTT,

25
Q

what is diagnostic for aHUS

A

absence of other diseases negative culture no TTP criteria C3 and C4 measurements, factors H,I,B, MCP, and antibody for factor H

26
Q

treatment for aHUS

A

plasma therapy Eculizumab (neutralizing Ab to C5) supportive therapy

27
Q

Glansmans thrombopenia

A

Gp2b3a defect, autosomal recessive

28
Q

Bernards soulier syndrom

A

Gp1b deficiency, autosomal recessive

29
Q

how does alcohol affect platelets

A

B12 or B9 deficiency can lead to lower platelets, they need nutrients to be created can be toxic to BM

30
Q

what are platelets

A

small non nucleated blood cells derived from bone marrow megakaryocytes

31
Q

what do alpha granules contain

A

procoagulant factor V Fibrinogen vWF PF4 Growth factors

32
Q

what do dense granules of platelets contain

A

ADP serotonin calcium

33
Q

what is this

A

normal peripheral blood smear

34
Q

what is bernard soulier syndrome

A

mutation of Gp1b

35
Q

what is glanzmans thrombasthenia

A

mutation of glycoprotein 2b3a

36
Q

what is this and what are the arrows pointing to

A

normal bone marrow

megkaryocytes

37
Q

normal platelet count

A

150,000-400,000

38
Q

platelet function tests

A

PFA-100

Platelet aggregation test

bleeding test

39
Q

what does PFA-100 do

A

screening test of platelet function that measures platelet adhesion and aggregation

40
Q

if collagen/epinephrine test result is abnormal what does that mean

A

could be due to aspirin resistance

41
Q

if only collagen/epinephrine test is normal then

A

platelet dysfunction exists

42
Q
A
43
Q

it the collagen/epinephrine test is abnormal what happens

A

you perform APD collagen test to confirm, it that is abnormal too then platelet dysfunction probably exists

44
Q

platelet aggregation test measure what

A

ability of various agonists to platelets to induce in vitro actvaton

45
Q

how does the platelet aggregation test work

A

when agonist is added the platelets aggregate and absorb less light so the transmission increases and it is detected by the photocell

46
Q

causes of thrombocytopenia

A

decreased platelet production/survival

sequestration

dilution

47
Q
A

thrombocytopenia

48
Q
A

lymphoma

49
Q
A

metastatic carcinoma

50
Q
A

aplastic anemia

51
Q
A

TTP

note: schistocytes

52
Q

symptoms of thrombocytopenia

A

petechiae and ecchtmoses

gum and nose bleeding

GI bleeding, hematuria, excessive menstrual flow

Subarachnoid hemorrhage and intracerebral hemorrhage rare but serious

53
Q
A

petechiae

IBI Test 4 (12 decks)

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