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August 24, 1963

Coexistence of von Willebrand's Disease and Hemophilia in a Family

Author Affiliations


From the Department of Biochemistry, Marquette University School of Medicine. Dr. Adlam is an Attending Physician, Veterans Administration Hospital, Wood, Wis.

JAMA. 1963;185(8):635-639. doi:10.1001/jama.1963.03060080031009

In the family of a woman who has von Willebrand's disease and who is a hemophilia carrier, two daughters have hemophilic sons. One of her sons, two daughters, and one granddaughter have von Willebrand's disease. None is known to have inherited both diseases. The coexistence in one individual of the hemophilic carrier state and von Willebrand's disease does not appear to accentuate the bleeding tendency.

Hemophilia was diagnosed by the prolonged clotting time and the defective prothrombin consumption time, with both the basic and the modified (with added hemolysate and aged serum) methods. Von Willebrand's disease was diagnosed by the type of bleeding, the hereditary pattern of an autosomal dominant, a prolonged bleeding time, and a positive tourniquet test. In both diseases, good therapeutic results were obtained by transfusions of fresh frozen plasma.