Antibodies to von Willebrand factor in von Willebrand disease

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The occurrence of an alloantibody directed against von Willebrand factor in a multitransfused patient with severe (type III) von Willebrand disease was first reported in 2 consecutive studies by Sarji et al. (1974) and Stratton et al. (1975). After this, 14 additional cases of alloantibodies were described and reviewed by Mannucci and Mari (1984). A survey carried out on behalf of the World Federation of Hemophilia identified 6 additional cases that, added to the 15 cases previously reported, bring to 21 the total number of patients reported so far with anti-von Willebrand factor alloantibodies (Mannucci and Cattaneo, 1991). In the frame of the survey, Hanna (1989) reported an antibody directed towards factor VIII (F.VIII) in a girl with severe von Willebrand disease. Since this antibody did not apparently react with von Willebrand factor, it will not be reviewed here.