PT and aPTT are within normal limits.
Platelet function test shows failure to aggregate to ristocetin.
Platelet function test shows failure to aggregate to ristocetin.
Agonists Ristocetin
![Picture](/uploads/2/5/3/2/25327069/1453392691.png?250)
Normal Patient
Diagnosis: Bernard-Soulier Syndrome
Rare autosomal recessive coagulopathy
Adhesion problem of platelet to vWF
defect GP1b/V/IX (CD42)
large giant platelet w pseudonucleolus
thrombocytopenia
Rare autosomal recessive coagulopathy
Adhesion problem of platelet to vWF
defect GP1b/V/IX (CD42)
large giant platelet w pseudonucleolus
thrombocytopenia
Jonathan G. Drachman. Inherited thrombocytopenia: when a low platelet count does not mean ITP. Blood. 2004;103: 390-398