Von Willebrand disease in Sweden: demography and treatment.

Registration of patient with von Willebrand disease in Sweden is ongoing but far from completed. Forty-seven patients with type 3 are diagnosed with coagulation assays. DNA studies performed at Karolinska Institute have shown that 21 type 3 probands were homozygous with the same mutation on the 2 alleles. If considering von Willebrand disease inherited as an autosomal dominant trait the number of heterozygous is calculated to 27000. However, the mode of inheritance is not yet settled. Treatment with desmopressin + tranexamic acid in type 1 is highly efficient, as is the blood factor concentrate Haemate (Centeon) in patients with type 3. Treatment in patients with type 2 must be individualized and is dependent on the response to desmopressin.