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.