7.15/16 Bleeding disorders

Question 1

antibody involved in neonatal alloimmune thrombocytopenia

Answer 1

HPA-1a antibody

Question 2

first child often affected in

Answer 2

neonatal alloimmune thrombocytopenia

Question 3

treatment for neonatal alloimmune thrombocytopenia

Answer 3

IVIG

corticosteroids

platelet transfusion from mom

Question 4

receptor for vWF

Answer 4

GPIb

Question 5

receptor for fibrinogen

Answer 5

GPIIb/IIIa

Question 6

dilution thrombocytopenia cause by

Answer 6

trauma / complicated surgery –> platelets are diluted by saline, red cells, plasma infusion

Question 7

what happens to platelets in ITP

Answer 7

IgG binds to platelet, then removed by macrophages

Question 8

young female usually has what bleeding disorder

Answer 8

ITP

Question 9

ITP bone marrow

Answer 9

increased megakaryocytes

Question 10

first line treatment for ITP

Answer 10

corticosteroids

IVIG

anti-D (WinRho) if Rh(D) positive

Question 11

follows viral illness in children

Answer 11

acute immune thrombocytopenia in children

Question 12

HIV associated thrombocytopenia caused by

Answer 12

HIV attacking megakaryocytes

Question 13

cause of neonatal thrombocytopenia

Answer 13

transplacental passage of maternal autoantibody

Question 14

end result of HIT

Answer 14

thrombin formation, which converts fibrinogen to fibrin clot

Question 15

lab tests for HIT

Answer 15

immunoassays to detect HIT antibodies

serotonin release assay

Question 16

treatment for HIT

Answer 16

direct thrombin inhibitors (lepirudin, argatroban)

Question 17

pentad of TTP

Answer 17

thrombocytopenia
MAHA
renal dysfunction
neuro disturbances
fever

Question 18

TTP caused by decreased activity of

Answer 18

ADAMTS-13 (breaks down sticky ultra large multimers)

Question 19

treatment for TTP

Answer 19

plasma exchange

maybe rituximab

Question 20

HUS vs TTP

Answer 20

prominent acute renal failure

no neuro sxs

more frequent in kids

normal ADAMTS-13

caused by e coli

not treated by plasma exchange

Question 21

PFA-100 good for detecting

Answer 21

meds that affect platelets

vWD

inherited platelet disorders

Question 22

both Bernard Soulier and Glanzmann thrombasthenia are inherited…

Answer 22

autosomal recessive

Question 23

Glanzmann thrombasthenia lacks aggregation to

Answer 23

ADP
collagen
Epinephrine

Question 24

Bernard Soulier lacks aggregation to

Answer 24

ristocetin

Question 25

Hermansky-Pudlak syndrome

Answer 25

albinism and gamma-storage pool disease (bleeding)

Question 26

to test for aspirin, add what to tube

Answer 26

arachidonic acid

Question 27

to test for clopidogrel, add what to tube

Answer 27

ADP

Question 28

to test for vWD, add what to tube

Answer 28

ristocetin

Question 29

to test for Bernard Soulier, add what to tube

Answer 29

ristocetin

Question 30

if tissue factor was in citrate tube (contaminant), does what to clotting time

Answer 30

decreases it (tissue factor causes clots)

Question 31

if heparin was in citrate tube (contaminant), does what to clotting time

Answer 31

increases it

Question 32

what to rx if bleeding due to liver disease

Answer 32

fresh frozen plasma

Question 33

how does liver disease affect clotting

Answer 33

decreases synthesis of vitamin K dependent factors (so increases PT and PTT)

Question 34

vWD inheritance

Answer 34

autosomal dominant for types 1 and 2 autosomal recessive for type 3

Question 35

if have vWD, will also decrease factor

Answer 35

8

Question 36

variant of vWD that has abnormal vWF

Answer 36

type 2

Question 37

best screening and diagnostic test for vWD

Answer 37

PFA-100 vWF activity (with ristocetin)

Question 38

hemophilia clinical severity correlates with

Answer 38

F8 activity level

Question 39

treatments for vWD

Answer 39

DDAVP (if mild) vWF concentrate anti-fibrinolytics

Question 40

treatment if hemophilia A develops alloantibody to F8

Answer 40

large doses of F8 recombinant 7a (novoseven)

Question 41

what test determines strength of F8 inhibitor (and dictates what treatment to use)

Answer 41

bethesda titer

Question 42

clinical manifestations of DIC

Answer 42

bleeding (obstetric, trauma) thrombosis (cancer)

Question 43

lab values for DIC: ``` platelet D-dimer PT PTT thrombin time fibrinogen ```

Answer 43

``` platelet: decr D-dimer: incr PT: incr PTT: incr thrombin time: incr fibrinogen: decr ```

Question 44

rx management of dic

Answer 44

transfusion anticoagulants fibrinolytic inhibitors (aminocaproic acid)