Bleeding Disorders Flashcards Preview

SBM > Bleeding Disorders > Flashcards

Flashcards in Bleeding Disorders Deck (44)
Loading flashcards...
0

vWF function

tethers platlets to subendo in vascular injury-->localizes and holds factor 8 to site of injury

1

genetics of vWF

autosomal dominant

2

vWF type 1

most common
low levels of vWF activity

3

vWF type 2

normal levels of vWF protein, but reduced activity due to abnormal molecule or decreased high molecular weight multimers

4

vWF type 3

absent vWF activity

5

aspirin and bleeding disorder

makes it worse!

6

lab testing in vWF

prolonged aPTT but normal PT
decreased or normal factor VIII fx
prolonged PFA-100
abnormal ristocetin test

7

DDAVP

desmopression;synthetic vasopression
stimulates release of vWF and favtor VIII from endothelial cells
good for type I, but not really type II because if no functional protein doesnt matter if you pump out more

8

other treatments for vw def

vw factor replacement- humate P, alphanate, cryoprecipitate
antifibronlytic therapy
contraceptives
medic alert bracelets

9

x linked recessive hemophilia

hemo A
hemo B

10

autosomal recessive hemophilia

hemo C
parahemophilia

11

heterozygotes of hemo

will have low levels but will generally not bleed (except maybe C)

12

hemo A

factor VIII def

13

hemo B

factor IX def (christmas disease)

14

hemo C

factor XI def

15

parahemophilia

factor V def

16

factor levels for different severities

mild: 5-40%
moderate 2-5%
severe: less than or equal to 1%

17

joint disease in hemophilia

bleeding into joing-->Fe causes synovial proliferation--> chronic synovitis-->arthritis-->joint destruction

18

labs in hemo

aptt prolonged

19

treatment of hemo

coagulation factor concentrations on demand
prophylaxis to reduce bleeding episodes

20

complications with hemophilia

chronic arthropathy
infectious diseases- hiv, hep c
inhibitors to missing coag factors (can complicate treatment)

21

Coagulation Factor Inhibitor disease

acquired hemophilia- spontaneous autoantibody to factor VIII

22

lab studies in aquired hemophilia

aPTT will be prolonged, but will NOT correct with mixing study
bethesda assay is used to measure antibody titer: if less than or equal to 5 BUs, may correct temporarily

23

treatment methodology of bleeding disorders

treat bleeding and do something about antibody

24

if low BU titer,

treat with high doses of factor VIII concentrate

25

if titer high

treat with bypass agent
--recombinant factor VII, FEIBA, porcine factor VIII

26

you can also do this for acquired hemophilia

immunosuppresion

27

hereditary fibrinolytic defects

deficient inhibitors
overexpression of plasminogen activator

28

deficinet inhibitor diseases

alpha 2 antiplasmin
plasminogen activator inhibitor deficiency

29

overexpression of plasminogen activator

quebec platelet disorder