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Blood, 1946, Vol. 1, No. 6, pp. 504-533.
© 1946 American Society of Hematology, Inc.

PSEUDOHEMOPHILIA

S. ESTREN M.D.1, L. SANCHEZ MÉDAL M.D.1, and WILLIAM DAMESHEK M.D.1

1 Blood Laboratory of the J. H. Pratt Diagnostic Hospital and Boston Dispensary and the Department of Medicine, Tufts College Medical School.

1. Eleven cases of a hemorrhagic diathesis are presented characterized by an increased bleeding time in the presence of a normal coagulation time, normal blood platelet count, and normal clot retraction. An analysis is made of 62 similar cases in the literature.

2. These cases, which have been designated as pseudohemophilia, probably represent a particular disorder of the capillaries, in which capillary retractility following trauma may be inherently defective.

3. The differential diagnosis of these cases from other types of hemorrhagic disease and the necessity for their recognition particularly from the prophylactic standpoint are stressed. Except for easily accessible local bleeding, therapy is at present ineffectual.

4. Standardized methods for the performance of hemorrhagic tests are suggested. The Lee-White method for the coagulation time may give normal values as high as 20 minutes or more.


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