Hemorrhagic Disorders Flashcards
two functions of VWF
- helps platelets stick to injured subendothelium
- carries and protects factor 8 around circulation
most common inherited bleeding disorder is
- von Willebrand disease
inheritance of vWD
- autosomal codominant
vWD caused by
- deficiency or synthesis of dysfunctional form of vWF
Type 1 vWD
- quantitative disorder
- 30% normal levels
VWD clinical manifestations
- mucocutaneous bleeding
- excessive bleeding after surgery
VWD excessive bleeding after surgery where
- mucosal surfaces
VWD mucocutaneous bleeding where
- heavy menstrual
- easy bruising
- GI bleeding
- nosebleeds
VWD excessive bleeding after which surgeries
- tooth extraction
- tonsillectomy
- hysterectomy
VWD joint and muscle bleeding?
- none!
most common symptom in women with VWD
percent affected
- menorrhagia (heavy mentrual bleeding)
- 75-95%
after length of time from first bleeding episode to diagnosis of VWD in women
- 17 years
VWD levels increased in
- pregnancy
- estrogen in OCP and HRT
effect of Type O blood on VWF
- makes VWF low
diagnosing VWD
- personal history of abnormal bleeding
- family history of abnormal bleeding
- low levels of VWF
how low does VWF need to be for VWD
- < 30%
treatment of VWD
- hormone manipulations
- desmopressin
- antifibrinolytics
- blood products and clotting factors
desmopressin MOA for treatment of VWD
- releases factor 8 and VWF from endothelial stores
desmopressin effects with prolonged use in VWD
- hyponatremia
antifibrinolytics MOA in VWD
- stabilize clot on mucosal surfaces
blood products and clotting factors in VWD treatment contain
- Factor 8 and VWF
hemophilia A is deficient in
- factor 8
hemophilia B is deficient in
- factor 9
hemophilia A and B inheritance
- X linked
