Response to infusions of polyelectrolyte fractionated human factor VIII concentrate in human haemophilia A and von Willebrand's disease

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Abstract
Factor VIII was purified from cryoprecipitate by ion exchange chromatogrpahy on solid phase polyelectrolyte E-5 (PE-E5). The product was highly purified (3.5 U VIII:C/mg protein) compared to conventional concentrate (0.3 U VIII:C/mg protein) with low fibrinogen, low isoagglutinin titer and a ratio of factor VIII coagulant activity (VIII:C) to factor VIII related antigen (VIIIR:Ag) of 16:1. Trial infusions of this material (PE VIII) were given to 3 patients with severe hemophilia A and 1 patient with homozygous von Willebrand''s disease. These patients also each received separate infusions of intermediate purity concentrate (IPC) for comparison. There were no adverse effects. The mean half-life of VIII:C after PE VIII infusion in the hemophiliacs was 10.9 h and after IPC was 12.1 h, a statistically insignificant difference. The survival of factor VIII coagulant antigen (VIII:CAg) was similar to that of VIII:C. The half-life of VIII:C and of VIII:CAg was very short after infusion of PE VIII in the patient with von Willebrand''s disease (2.4 h). IPC when infused in this patient produced a typical secondary rise of VIII:C. Two bleeding episodes in severe hemophiliacs were satisfactorily treated with PE VIII. PE-E5 deserves further study as a means of preparing clinical concentrates of factor VIII.