Consensus Diagnosis and Treatment of von Willebrand Disease.

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  • ｖｏｎ　Ｗｉｌｌｅｂｒａｎｄ病の診断と治療

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Abstract

The population of patients with von Willebrand disease (vWD) in England, Italy, and Canada were 9.3, 3.6, and 3.1/105 persons, respectively, in 1998. In contrast, the number in Japan has increased gradually up to 735 cases (0.56/105 persons), possibly, in parallel with the extent of concern about vWD. To normalize criteria for a diagnosis of vWD worldwide, the ISTH/SSC introduced a guideline for Type 1 vWD that consists of significant bleeding symptoms, laboratory findings, and inheritance. The significant bleeding symptoms include frequent and severe nose bleeding, menorrhagia, and abnormal bleeding after tooth extraction and operations. The laboratory finding shows low vWF under <2 SD from mean values of vWF : Ag and vWF : RCo, corresponding to blood groups. Then, positive family history requires at least one first-degree relative or at least two second-degree relatives who have significant bleeding symptoms and compatible laboratory data to vWD. The treatment of mild vWD involves an administration of the synthetic vasopressin analog DDAVP. On the other hand, in severe cases, including Type 3, 2B, and 2N vWD, FVIII/vWF complex concentrate would be required for the replacement therapy. Especially, at delivery, the preventive administration of FVIII/vWF for after-bleeding or hematoma should be recommended in Types 2 and 3 vWD.

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