hemorrhagic disorders Flashcards
two functions of vWF
- adhesion of platelets to endothelial cells
2. binds factor VIII in circulation and protects it
treatment for Von Willebrand Disease
- hormones- help with heavy menstrual bleeding
- desmosomes- help release vWF and FVIII from endothelial cells
- anti-fibrinolytics- helps stabilize clot on mucosal lining
- blood products containing clotting factors VIII and vWF
lab results of Von Willebrand Disease
- normal PT, aPTT, and TT
- low vWF antigen and activity and factor VIII level
- PFA-100 level will be prolonged
clinical manifestations
- menorrhagia
- abnormal mucocutaneous bleeding
- GI bleeding
- epistaxis
- heavy abnormal bleeding post surgery
what type of inheritance pattern is von willebrand disease?
autosomal co dominant
why is von willebrand disease difficult to diagnose?
factors like stress, anxiety, and menstrual cycle effect levels of vWF, ask about family history and history of abnormal bleeding
T/F people with von willebrand disease experience joint and muscle bleeding
false
______ is found in 75-95% of women with von willebrand disease
menorrhagia
average length of time from first bleeding experience to diagnosis is ____ years
17
what is the most common inherited bleeding disorder?
von willebrand disease
hemarthrosis, joint pain, edema, bleeding into muscle, GU bleeding, CNS bleeding is associated with
hemophilia A and B
hemophilia A is associated with what factor
8
hemophilia B is associated with what factor
9
what would a lab for a patient who has hemophilia show?
normal PT and TT
prolonged aPTT
normal PFA-100
normal vWF antigen and activity
< 1% of either factor 8 or 9 results in
spontaneous bleeding
adding factor 8 and 9 results in
cumulative affect where risk of bleeding decreases
treatment for hemophilia
- DDAVP for hemophilia A where it can raise VIII 3 fold before surgery
- transfusions replace deficit
what disease are people with hemophilia at risk due to the treatment?
HIV- hepatitis C
orthopedic complication of hemophilic patients
- recurrent joint bleeds
- cartilage damage
- accelerated arthritis
- muscle wasting
- need for joint replacement
Hemophilia show what type of inheritance pattern ?
x-linked
Hemophilia is x-lnked recessive… how would women have hemophilia?
- two parents who are carriers
- lyonization
- spontaneous mutation
- turners syndrome
factor XI deficiency
- autosomal recessive
- Ashkenazi Jews
- NO FACTOR CONCENTRATE
- treat with plasma
- post-surgical bleeding and mucocutaneous bleeding
