Bleeding Disorders

Question 0

genetics of vWF

Answer 0

autosomal dominant

Question 1

vWF function

Answer 1

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

Question 2

vWF type 1

Answer 2

most common

low levels of vWF activity

Question 3

vWF type 2

Answer 3

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

Question 4

vWF type 3

Answer 4

absent vWF activity

Question 5

aspirin and bleeding disorder

Answer 5

makes it worse!

Question 6

lab testing in vWF

Answer 6

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

Question 7

DDAVP

Answer 7

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

Question 8

other treatments for vw def

Answer 8

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

Question 9

x linked recessive hemophilia

Answer 9

hemo A

hemo B

Question 10

autosomal recessive hemophilia

Answer 10

hemo C

parahemophilia

Question 11

heterozygotes of hemo

Answer 11

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

Question 12

hemo A

Answer 12

factor VIII def

Question 13

hemo B

Answer 13

factor IX def (christmas disease)

Question 14

hemo C

Answer 14

factor XI def

Question 15

parahemophilia

Answer 15

factor V def

Question 16

factor levels for different severities

Answer 16

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

Question 17

joint disease in hemophilia

Answer 17

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

Question 18

labs in hemo

Answer 18

aptt prolonged

Question 19

treatment of hemo

Answer 19

coagulation factor concentrations on demand

prophylaxis to reduce bleeding episodes

Question 20

complications with hemophilia

Answer 20

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

Question 21

Coagulation Factor Inhibitor disease

Answer 21

acquired hemophilia- spontaneous autoantibody to factor VIII

Question 22

lab studies in aquired hemophilia

Answer 22

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

Question 23

treatment methodology of bleeding disorders

Answer 23

treat bleeding and do something about antibody

Question 24

if low BU titer,

Answer 24

treat with high doses of factor VIII concentrate

Question 25

if titer high

Answer 25

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

Question 26

you can also do this for acquired hemophilia

Answer 26

immunosuppresion

Question 27

hereditary fibrinolytic defects

Answer 27

deficient inhibitors | overexpression of plasminogen activator

Question 28

deficinet inhibitor diseases

Answer 28

alpha 2 antiplasmin | plasminogen activator inhibitor deficiency

Question 29

overexpression of plasminogen activator

Answer 29

quebec platelet disorder

Question 30

acquired fibrinolytic defects

Answer 30

cancer liver disease DIC local relase plasminogen activators from prostate surgery

Question 31

lab results of fibrinolytic diseases

Answer 31

prolonged PTT and PT | increased d-dimers

Question 32

treatment of fibrinolytic disorders

Answer 32

``` supportive with plasma infusions antifibrinolytic drugs (if NO DIC) ```

Question 33

antifibrinolytic drugs

Answer 33

epsilon aminocaproic acid | tranexamic acid

Question 34

DIC labs

Answer 34

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

Question 35

treatment DIC

Answer 35

plasma to replace coagulation factors and anticoagulants cryoprecipitate (to replace fibrinogen) platelets, RBC **heparin will stop DIC, but may lead to bleeding

Question 36

liver disease parameters

Answer 36

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

Question 37

liver disease labs

Answer 37

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

Question 38

treatment of liver disease

Answer 38

transplant; everything else is only temporary

Question 39

vitamin K is required for

Answer 39

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

Question 40

ways to get vitamin K def

Answer 40

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

Question 41

lab studies vitamin k def

Answer 41

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

Question 42

treatment vitamin k def

Answer 42

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

Question 43

must give vitamin K concurrently because

Answer 43

short half life of factor VII in plasma