Bleeding disorders Flashcards
Bleeding that is:
- superficial
- petechiae
- spontaneous
Platelet bleeding
Bleeding that is:
- deep joints
- Big bleeds
- Trauma
Factor bleeding
Most common hereditary bleeding disorder
Autosomal dominant
Variable severity
Von Willebrand Disease
**vW factor decreased or abnormal
vW factor made where?
megakaryocytes and subendothelial cells
vWF carries what factor?
VIII
Three types of vW disease:
Type 1 (70%) : decreased vWF
Type 2 (25%) : abnormal vWF
Type 3 (5%) : no vWF
Sx of vW disease?
mucosal bleeding
deep joint bleeding (severe cases)
What test will be NORMAL in a pt with vW disease?
INR
Platelet membrane protein that binds vWF?
GP Ib
Tx fro vW disease?
DDAVP (for type I)
Cryoprecipitate (contains vWF and VIII)
Factor VIII
X-linked recessive bleeding disorders:
hemophilia A and B
most common FACTOR deficiency
factor VIII decreased
hemophilia A
common sx of hemophilia A:
deep joint bleeding
prolonged bleeding after dental work
NORMAL lab results in hemophilia A and B?
INR
TT
Platelet count
bleeding time
What does PTT look like in hemophilia?
prolonged (low factor VIII or IX)
**corrects with mixing study
Tx of hemophilia A:
DDAVP
Factor VIII
What is the difference between hemophilia A and B
A = VIII problem
B = IX problem, MUCH LESS COMMON
Rare; bleeding only after trauma:
XI deficiency
severe neonatal bleeding
XIII
Crosslinks fibrin –> clot
XIIIa
Abnormal GP Ib
Big platelets
Severe Bleeding
Bernard-Soulier Syndrome
No IIb-IIIa
No aggregation
Severe bleeding
Glanzmann Thrombasthenia
No alpha granules in platelets
Big, empty platelets
Mild bleeding
Gray Platelet Syndrome
Platelet prob related to Chediak-Higashi?
delta Granule deficiency
