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December 1977

Hemophilia in Females: Use of Immunologic AHG-Determination in Its Diagnosis

Author Affiliations

From the Department of Medicine, University of Illinois Hospital, Chicago.

Arch Intern Med. 1977;137(12):1715-1716. doi:10.1001/archinte.1977.03630240049016

Because of its rarity, the diagnosis of "classical" hemophilia (factor VIII "deficiency," hemophilia A) in a female has always been established with a certain apprehension and requires the exclusion of the von Willebrand's syndrome. The biological characteristics of these diseases has recently advanced considerably and firmer criteria for differentiating these diseases include the determination of both the procoagulant and immunological activity of factor VIII.

REPORT OF A CASE  A 53-year-old woman was referred to the University of Illinois (Chicago) Hospital in 1975 because of the inability to walk that resulted from bilateral Morton's neuromata and a long-standing history of bleeding. The bleeding history began at age 5 and included frequent bruises, epistaxis, and occasional hemarthrosis. Severe menorrhagia led to sterilization by radiation at age 41. Transfusions after severe bleeding were required after hemorrhoidectomy (1955) and tooth extractions (1974). The hemorrhoidectomy procedure required 60 units of blood.The family history disclosed

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