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Flashcards in Bleeding Disorders Deck (44):
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)

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hemo C

factor XI def

15

parahemophilia

factor V def

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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

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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

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treatment methodology of bleeding disorders

treat bleeding and do something about antibody

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if low BU titer,

treat with high doses of factor VIII concentrate

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if titer high

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

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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

30

acquired fibrinolytic defects

cancer
liver disease
DIC
local relase plasminogen activators from prostate surgery

31

lab results of fibrinolytic diseases

prolonged PTT and PT
increased d-dimers

32

treatment of fibrinolytic disorders

supportive with plasma infusions
antifibrinolytic drugs (if NO DIC)

33

antifibrinolytic drugs

epsilon aminocaproic acid
tranexamic acid

34

DIC labs

prolonged PT, aPTT
elevated d-dimers
decreased fibrinogen, platlet count, rbcs, antithrombin, protein c, protein S
presence of schistocytes

35

treatment DIC

plasma to replace coagulation factors and anticoagulants
cryoprecipitate (to replace fibrinogen)
platelets, RBC

**heparin will stop DIC, but may lead to bleeding

36

liver disease parameters

bleeding due to decreased synthesis of coagulation factors, fibronlytic factor inhibitors

clotting due to decreased clearance of activated coagulation factors

37

liver disease labs

prolonged PTT and PT, but this doesnt correspond with bleeding
low factor levels except VIII

38

treatment of liver disease

transplant; everything else is only temporary

39

vitamin K is required for

gamma-carboxylation of coagulation factors which improves coagulation factor binding to phospholipid surface of platelet

40

ways to get vitamin K def

dietary-poor intake, malabsorption due to pancreatic or small-bowel disease, alcoholism
medications
warfarin!

41

lab studies vitamin k def

prolonged PT, PTT
factor 2, 7, 9, 10 decreased
normal 5 (so we know it's not liver disease)

42

treatment vitamin k def

give vitamin K
fresh frozen plasma can get you out of a quick bind, but not long lasting

43

must give vitamin K concurrently because

short half life of factor VII in plasma