314560
VON WILLEBRAND DISEASE, X-LINKED FORM


TEXT
Holmberg and Nilsson (1973) used a monospecific precipitating rabbit antiserum against human antihemophilic factor-related protein to study 77 patients with von Willebrand disease (defined by low factor VIII, prolonged bleeding time and decreased platelet adhesiveness). They found two groups of patients. The larger group (57 patients) corresponded to classic von Willebrand disease and had low factor VIII protein. The other 20 patients had normal amounts of protein. Their disorder may be X-linked dominant. Infusion of human antihemophilic factor containing fraction I-O did not produce the delayed increase in antihemophilic factor characteristic of the first group. Various forms were suggested also by Koutts et al. (1975).

REFERENCES
1. Holmberg, L., Nilsson, I. M. Two genetic variants of Von Willebrand's disease. New Eng. J. Med. 288: 595-598, 1973. [PubMed: 4539979, related citations] [Full Text: Atypon, Pubget]

2. Koutts, J., Meyer, D., Rickard, K., Scott, L., Firkin, B. G. Heterogeneity in biological activity of human factor VIII antibodies. Brit. J. Haemat. 29: 99-107, 1975. [PubMed: 1081403, related citations] [Full Text: Pubget]

Creation Date: Victor A. McKusick : 6/4/1986
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