Immunologic differentiation of classic hemophilia (factor 8 deficiency) and von Willebrand's dissase, with observations on combined deficiencies of antihemophilic factor and proaccelerin (factor V) and on an acquired circulating anticoagulant against antihemophilic factor.

Heterologous antiserum was prepared in rabbits against highly purified human antihemophilic factor (AHF, factor VIII). This antiserum blocked the clot-promoting properties of AHF and, when suitably absorbed, formed a single precipitin line against AHF upon immunoelectrophoresis. Material antigenically similar to normal AHF was detected in normal amounts in plasma concentrates in each of 22 patients with classic hemophilia, in a patient with an acquired circulating anticoagulant against AHF, and in a patient with deficiencies both of AHF and proaccelerin (factor V). AHF-like antigen was present in normal human serum. In contrast, material antigenically related to AHF was found in decreased amounts in the concentrates prepared from the plasma of 11 patients with von Willebrand's disease. The experiments described suggest that von Willebrand's disease is a disorder in which a true deficiency of AHF exists. Whether the AHF-like material found in classic hemophilia is nonfunctional through a defect in structure or through the intervention of an inhibitor has not been shown.