The treatment of hemophilia: past tragedy and future promise

Article Abstract:

Since 1965 patients with hemophilia have been treated with concentrated preparations of blood products which contain essential coagulation factors which they lack. Use of factor VIII and other blood- derived products changed the outlook for many of these patients and for the first time gave them relief from their repeated bouts with bleeding. However, it slowly became clear that when blood from many individuals is pooled in order to prepare the necessary concentrates, infection from a single donor could be carried to all recipients. Early on, nearly all patients with hemophilia became infected with one form or another of hepatitis, and although serious itself, chronic liver disease was considered an acceptable price for relief from their bleeding. The situation with AIDS is even more devastating and it is now increasingly clear that given enough time individuals who receive blood contaminated by human immunodeficiency virus, HIV, will eventually develop AIDS. An article by Schimpf and others in the New England Journal of Medicine shows that the contamination of blood products can be eliminated by a pasteurizing process that removes the risk of HIV while maintaining the biologic activity of the product. This technique could have been used earlier except for concern with the cost of providing such material. The scale of the pooling process needs to be imagined to truly understand the magnitude of the risk: as many as 20,000 donors are required to make a single batch of coagulation factor VIII. The life of the hemophiliac has been made even more difficult by the realization that there is a severe risk of developing AIDS for many of them.

Research, editorial

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Absence of anti-human immunodeficiency virus types 1 and 2 seroconversion after the treatment of hemophilia A or von Willebrand's disease with pasteurized factor VIII concentrate

Article Abstract:

Patients with hemophilia, or bleeders, are often successfully treated with clotting factors that are produced from large quantities of pooled human blood. Careful screening and preparation techniques have largely removed spirochetes (syphilis) and bacteria from the pooled blood. However, viral hepatitis and human immunodeficiency virus (HIV), the virus responsible for AIDS (acquired immunodeficiency syndrome), have been spread by the use of blood and blood products. Pasteurization of blood products at 60 degrees C for a period of 10 hours led to a complete elimination of these viruses in a population of 155 patients with hemophilia at 11 treatment centers. The patients had been screened for both HIV type I and II by a test known as enzyme-linked immunosorbent assay (ELISA), and positive results were confirmed by Western blot, an assay of the incorporation of the HIV virus into the patients' genetic material. Absence of infection in these patients treated by pasteurized factor VIII was compared with the rate of infection in a group of control patients who had been previously treated with unpasteurized blood products. The rate of infection of this group varied with total dose of blood products received, but in total more than 50 percent of the group became infected with AIDS. This study clearly establishes that the treatment of hemophilia with factor VIII concentrate which has been pasteurized is safe and appears free from the risk of HIV.

Measurement, HIV antibodies

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