title: Re-evaluation of the therapeutic efficacy of DDAVP in type IIB von Willebrand's disease.
abstract: With few exceptions, 1-desamino-8-D-arginine vassopressin (DDAVP) has been shown to be useful in securing haemostasis in patients with von Willebrand's disease (vWd). In type IIB vWd, DDAVP has been reported to have no beneficial effects and to be contraindicated because it causes or worsens thrombocytopenia, due to in vivo platelet aggregation. Nevertheless, it was previously demonstrated that DDAVP may have clinical utility in some patients with type IIB vWd. Additional findings obtained in seven type IIB vWd patients of different kindreds undergoing minor surgical procedures are now reported. It was observed that DDAVP corrected the bleeding time in every case, with effects lasting for 2 h. Mean platelet counts decreased 30 min after DDAVP to variable degrees, depending on the anticoagulant used for blood collection, but were normal 2 h later. Furthermore DDAVP normalized FVIII, von Willebrand factor (vWf) antigen (vWf:Ag), and to a lesser extent, vWf ristocetin cofactor activity (vWf:RCoF). Intermediate and large vWf multimers appeared after 30 min. There were no bleeding complications during or after surgery, nor evidence of thrombosis. It was thus confirmed that DDAVP has clinical utility in the prevention of bleeding symptoms in different type IIB vWd patients. Therefore, despite the transitory thrombocytopenia and the incomplete restoration of larger vWf multimers, the use of this drug should be reconsidered for patients with type IIB vWd.
authors.affiliation: University of Padua, Medical School, Institute of Medical Semeiotics, Italy.
authors.forename: A
authors.initials: A
authors.lastname: Casonato
authors.forename: E
authors.initials: E
authors.lastname: Pontara
authors.forename: D
authors.initials: D
authors.lastname: Dannhaeuser
authors.forename: A
authors.initials: A
authors.lastname: Bertomoro
authors.forename: M T
authors.initials: MT
authors.lastname: Sartori
authors.forename: P
authors.initials: P
authors.lastname: Zerbinati
authors.forename: A
authors.initials: A
authors.lastname: Girolami
mesh_terms: D003894:Deamino Arginine Vasopressin / Q000009:adverse effects / Q000627:therapeutic use*; D005169:Factor VIII / Q000378:metabolism; D006470:Hemorrhage / Q000517:prevention & control; D006801:Humans; D007431:Intraoperative Complications / Q000517:prevention & control; D007700:Kinetics; D010976:Platelet Count; D013921:Thrombocytopenia / Q000139:chemically induced; D014842:von Willebrand Diseases / Q000097:blood / Q000188:drug therapy*; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D014841:von Willebrand Factor; D005169:Factor VIII; D003894:Deamino Arginine Vasopressin