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Von Willebrand: The scientist, the disease, the factor, and the treatment

2007; Elsevier BV; Volume: 120; Linguagem: Inglês

10.1016/j.thromres.2007.03.009

1879-2472

Erik Berntorp, David Lillicrap,

Myeloproliferative Neoplasms: Diagnosis and Treatment

Von Willebrand disease (VWD) is a bleeding disorder caused by a dual defect in (i) platelet adhesion and aggregation due to reduced and/or dysfunctional von Willebrand factor (VWF) and (ii) secondary coagulation due to unstabilised factor VIII (FVIII) resulting in decreased plasma levels. This bleeding disorder was first described by a Finnish physician, Erik Adolf von Willebrand (1870–1949), who reported a new type of haemorrhagic disorder among people living on the Åland islands off the coast of Finland. Since its discovery, VWD has been extensively studied, and our understanding of the disease, the factor and its treatment has greatly improved.