Pseudo von Willebrand Disease is a rare disorder that is often misdiagnosed. GPIBA is the platelet ligand that von Willebrand factor binds to.
Inheritance: autosomal dominant
Gene affected: GP1BA = glycoprotein Ib (platelet), alpha polypeptide
(1) bleeding disorder with mucocutaneous bleeding, especially after aspirin or other drug with antiplatelet activity
(2) prolonged bleeding time and fluctuating thrombocytopenia
(3) increased response to low dose ristocetin in platelet-rich plasma (PPP)
(4) bleeding not corrected by cryoprecipitate
(5) platelet hyperaggregability (due to increased affinity of the receptor for vWF)
(6) bleeding corrected by transfusion of a platelet concentrate
(7) absence of mutations in the von Willebrand Factor gene
Differential diagnosis: type 2B von Willebrand disease (a defect in vWF results in increased affinity with a normal GP1BA)
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Specialty: Hematology Oncology, Clinical Laboratory