PROPHYLACTIC THERAPY FOR HEMOPHILIA IN A DEVELOPING-COUNTRY, TURKEY

Prophylaxis has been practiced for many years in Europe and is gaining acceptance worldwide with current viral inactivation procedures. Unfo rtunately, the high cost of praphylaxis is currently the major obstacl e to its implementation in developing countries such as Turkey. The ai m of this controlled preliminary study is to evaluate the efficacy, sa fety, and feasibility of prophylaxis. Seven boys aged 1.5-7 years (5.0 +/- 1.8) who had severe hemophilia (six A, one B) received 20-50 IU/k g factor twice weekly and were followed up for 6-24 months (14.5 +/- 6 .6). Intermediate concentrates have been used in hemophilia A and ultr apure product for hemophilia B. The data obtained for the same group o f patients before prophylaxis were used as a control group. Another co ntrol group was selected in another group of IO hemophiliacs, mean age 12.5, and received treatment on demand. During prophylactic treatment , the episodes of bleeding were decreased (from 10.5 +/- 3.2 to 4.5 +/ - 3.6). Orthopedic and radiologic joint scores were stable (from 0 to I and from 1.1 +/- 1.2 to 1.0 +/- 1.5). The patients spent significant ly fewer days in the hospital (from 18 +/- 12 to 0.7 +/- 0.6). None of the patients was infected with hepatitis A, hepatitis B, or human imm unodeficiency virus. One patient was seroconverted with anti-hepatitis C virus in the third month of prophylaxis. Mean consumption of concen trates for prophylaxis was 3489 +/- 960 IU/kg per year compared with 2 073 +/- 1302 in conventional therapy. Prophylaxis was superior to trea tment on demand even when given in a twice-weekly period with intermed iate concentrates. In Third World countries, prophylaxis should be tri ed at least in selected severely hemophilic children in order to preve nt disabilities.