[Long-term blood transfusion in sickle-cell anemia].

Ten homozygous sickle cell patients, aged from 4 to 20 years, underwent a periodic red blood cells transfusion program. Indications were = one stroke or more in 4 cases, repeated severe painful crisis in 2 cases, and for others = chronic anaemia induced by hypersplenism, priapism, chronic leg ulceration, dwarfism with delayed sexual maturation. Transfusion program lasted from 6 months to 6 years. In 7 cases, a phlebotomy preceded the transfusion by the same venipuncture in order to reduce the inevitable iron overload progression. Clinical benefit was clear in the following cases: lack of painful crisis with return to school, leg ulceration healing, growth and weight increase, onset of puberty. Benefit is more difficult to assess in the other observations and, particularly, in stroke. No red cell antibodies were found. An HIV contamination occurred in 3 children. All transfused patients during a period exceeding one year developed iron overload, needing a daily chelation by intramuscular deferoxamine in two of them.