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June 1956

Pseudohemophilia Type B: Hereditary Hemorrhagic Diathesis Characterized by Prolonged Bleeding Time and Decrease in Antihemophilic Factor

Author Affiliations


From the Department of Hematologic Research, Medical Research Institute, Michael Reese Hospital. The Department is supported in part by the Michael Reese Research Foundation and the Hulda B. & Maurice L. Rothschild Foundation for Scientific Research. Ruth Berger Reader Fellow, Hematology Research Foundation, 1954-1955 (Dr. Ramot).

AMA Arch Intern Med. 1956;97(6):715-725. doi:10.1001/archinte.1956.00250240067006

Recently, we observed a 13-year-old girl, the offspring of consanguinous parents, who exhibited a severe hemorrhagic diathesis. Laboratory studies revealed a prolonged bleeding time, normal platelet count, and poor prothrombin consumption. The thromboplastin-generation test and other assay procedures demonstrated a marked decrease in antihemophilic factor (AHF). A search of the literature suggested that this syndrome has been encountered by several investigators during the last two years.* So far we have been able to find 19 similar cases (14 females) in the literature, although most of the reports are rather fragmentary or of a preliminary nature.

This communication attempts to describe the clinical, hematologic, and genetic aspects of this disorder, which in our opinion represents a definite disease entity.

REPORT OF A CASE  A 13-year-old white Jewish girl was transferred to our department from another hospital for diagnosis and treatment of her severe hemorrhagic diathesis. She had had severe uterine bleeding

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