pseudo-von Willebrand's disease

pseudo-von Willebrand's disease

Platelet-type von Willebrand disease–vWD An AD condition similar to type IIB vWD, with moderately severe Sx Lab ↑ Bleeding time, ↓ von Willebrand factor–vWF and factor VIII levels, ↑ ristocetin-induced platelet aggregation, absence of large vWF multimers and presence of the same multimers in platelets Pathogenesis Unknown; may involve platelet glycoprotein IB Management Cryoprecipitate. Cf Von Willebrand's disease.
References in periodicals archive ?
Pseudo-von Willebrand's disease. An intrinsic platelet defect with aggregation by unmodified human factor VIII/ von Willebrand factor and enhanced adsorption of its highmolecularweight multimers.
The response to desmopressin therapy in patients with type 1 is usually good.[6] However, desmopressin is contraindicated in type 2 and pseudo-von Willebrand's disease because it can increase platelet aggregation and cause thrombocytopenia.[7] Consequently, It is important to know which type of von Willebrand's disease is present so that safe and effective therapy can be given.