Two Genetic Variants of von Willebrand's Disease

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Abstract
A monospecific precipitating antiserum against an antihemophilic-factor-related protein was developed in rabbits. Using an immunochemical technic, we found our 77 patients with von Willebrand's disease to fall into two separate genetic groups, 57 with a low, and 20 patients with a normal content of the protein, conceivably the von Willebrand factor. The major group corresponds to the classic form of von Willebrand's disease with an autosomal and dominant inheritance, but the second type may be X-chromosomally transmitted. In two patients in the second group, infusion of human antihemophilic factor containing fraction l-0 did not produce the retarded increase in antihemophilic factor characteristic of the first group. The second group thus appears to represent a separate disorder different from von Willebrand's disease and hemophilia A but with features resembling both of them.
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