A patient with Hageman factor (HF) deficiency is described. This syndrome is characterized by complete absence of any hemorrhagic tendency in the presence of laboratory findings which, as a rule, are associated with severe disturbances in the hemostatic mechanism. The clotting time was markedly prolonged, the plasma prothrombin time was normal, but prothrombin consumption was decreased. The thromboplastin generation test revealed that HF is essential for blood thromboplastin formation at least in vitro.
Procedures for the differentiation of HF deficiency from AHF, PTC and PTA deficiency syndromes are outlined.
Transfusions of as little as 50 cc. of 20 day old blood normalized the abnormal clotting tests immediately for a period of about 36 hours.
The basis for the apparent lack of need for preoperative preparation with blood transfusions in HF deficiency is discussed.