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Article
June 11, 1960

FATAL CASE OF CONGENITAL PLASMA THROMBOPLASTIN COMPONENT DEFICIENCYFAILURE OF RESPONSE TO THERAPY IN CHRISTMAS DISEASE

Author Affiliations

Fort Defiance; Charlottesville; Staunton, Va.

From King's Daughters Hospital, Staunton (Drs. Carter and Menk), and the Department of Clinical Pathology, University of Virginia Hospital, Charlottesville, Va.

JAMA. 1960;173(6):631-634. doi:10.1001/jama.1960.03020240019003
Abstract

There is little available information regarding the treatment of plasma thromboplastin component (PTC) deficiency (Christmas disease.) An 18-year-old man with this disease developed a hematoma which increased in size despite multiple whole blood and plasma transfusions. No inhibitors or antibodies against PTC were present, and as much as 3 liters of whole blood was administered in one 24-hour period. Nevertheless, the PTC concentration did not rise to a levelhigher than that found in the mildest untreated cases of the disease. The patient died in extreme dyspnea, and autopsy revealed an extensive retroperitoneal hematoma, hemoperitoneum, and left hemothorax. The levels of PTC concentration actually attained by the treatment given would be expected if equilibration of PTC between intravascular and extravascular spaces occurs and if the half-life does not exceed 24 hours. The amount of blood or plasma needed to produce a hemostatic level of PTC in the disease is probably equal to that required in classic hemophilia of the same degree of clinical severity.

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