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
hemophilia A and B found mostly in
- men
PT of hemophilia A
- normal
PT of type 1 VWD
- normal
aPTT of hemophilia A
- prolonged
aPTT of type 1 VWD
- normal
- slightly prolonged
TCT of hemophilia A
- normal
TCT of type 1 VWD
- normal
PFA-100 of hemophilia A
- normal
PFA-100 of type 1 VWD
- prolonged
factor 8 level of hemophilia A
- low
factor 8 level of type 1 VWD
- low
- not very low
VWF antigen hemophilia A
- normal
VWF antigen type 1 VWD
- low
VWF activity hemophilia A
- normal
VWF activity type 1 VWD
- low
spontaneous bleeding presents with a factor 8 or 9 level of
- <1%
hemophilia bleeding sites
- hemarthorsis
- bleeding into muscles
- CNS bleeding
- GU bleeding
- retroperitoneal bleeding
- oro- and nasopharynx
orthopedic complications of hemophilia
- recurrent joint bleeds
- synovitis
- cartilage damage
- accelerated arthritis
- need for joint replacement
treatment of hemophilia
- desmopressin
- replace deficient factor
what form of hemophilia do you use desmopressin for
- mild hemophilia A
hemophilia inhibitors
- patients with hemophilia that develop neutralizing antibodies to clotting factor concentrates
hemophilia inhibitors common in which form ion hemophilia
- A
women can have low levels of factor 8 or 9 due to a process called
- lyonization
what is lyonization
- one of the copies of X chromosome in females is inactivated
inheritance of factor 9 deficiency
- autosomal recessive
factor 9 deficiency most commonly found in which population
- Ashkenazi jews
