Correspondence
Hemophilia A
N Engl J Med 1994; 330:1617June 2, 1994
- Article
To the Editor:
Although Dr. Hoyer has written a comprehensive review of the medical aspects of hemophilia (Jan. 6 issue),1 the review does not address the effect on patients of blood products contaminated with the human immunodeficiency virus (HIV). The contamination of the blood supply with HIV was the greatest iatrogenic catastrophe in history. Over 7000 people with coagulation disorders were infected between 1979 and 1985, until virally inactivated clotting-factor concentrates were widely introduced2; over 2000 people have died already, and the remainder will eventually have total immune dysfunction3. Hundreds of sexual partners and children were unwittingly infected through sexual and vertical transmission3 before risk-reduction programs were established by the National Hemophilia Foundation, the Centers for Disease Control and Prevention, and the Maternal and Child Health Bureau. The lives of three generations of infected people and their families have been forever altered and, in many cases, emotionally and financially devastated.
Unfortunately, viral and protein contamination of clotting-factor concentrates continues to be tolerated. Both parvovirus B19 and hepatitis A virus can be transmitted by clotting-factor concentrates and are not inactivated by some viral-inactivation procedures, such as solvent-detergent treatment2. Are we risking a future catastrophe? Will people with hemophilia provide the next early warning that our nation's blood supply is not safe?
3 ReferencesGlenn F. Pierce, Ph.D., M.D.
National Hemophilia Foundation, New York, NY 100121
Hoyer LW . Hemophilia A. N Engl J Med 1994;330:38-47
Full Text | Web of Science | Medline2
Pierce GF ,Lusher JM ,Brownstein AP ,Goldsmith JC ,Kessler CM . The use of purified clotting factor concentrates in hemophilia: influence of viral safety, cost, and supply on therapy. JAMA 1989;261:3434-3438
CrossRef | Web of Science | Medline3
AIDS update: AIDS cases and surveillance as of June 30, 1993. Chapter advisory 190. Medical bulletin 186. New York: National Hemophilia Foundation, September 21, 1993.
To the Editor:
In his article on hemophilia, Hoyer cites “the effect of the disease on history through its presence in Queen Victoria's descendants.” Although the first accurate description of hemophilia in the medical literature is that of John Conrad Otto in 1803,1 this disease was recognized and discussed in the Talmud and other Hebrew writings nearly 2000 years ago2. The Talmud (Tractate Yebamot, folio 64b) states that if a woman circumcised her first child and he died as a result of exsanguination and a second child died similarly, she must not circumcise her third child. Furthermore, if two sisters circumcise their sons and both die from exsanguination, the third sister may not circumcise her son. The explanation offered is that members of some families have “loose blood,” whereas normally the blood is “held fast” (i.e., it coagulates). Discussion of this disease in the post-Talmudic rabbinical literature is detailed elsewhere3. The sages of the Talmud and subsequent rabbinical authorities had a remarkable knowledge of the heredity of a familial bleeding disorder, which was probably hemophilia.
3 ReferencesFred Rosner, M.D.
Mt. Sinai Services at Queens Hospital Center, Jamaica, NY 114321
Otto JC . An account of an hemorrhagic disposition existing in certain families. Med Repos 1803;6:1-42
Rosner F . Hemophilia in the Talmud and rabbinic writings. Ann Intern Med 1969;70:833-837
Web of Science | Medline3
Rosner F. Hemophilia in classic rabbinic texts. J Hist Med Allied Sci (in press).
