[Systematic blood transfusions in adult homozygote sickle-cell anemia. Study of 11 cases followed for 5 to 10 years].
Eleven patients (5 men and 6 women) with sickle-cell anaemia were treated with systematic repeated blood transfusions to avoid the sickling crises and complications of the disease and were followed up for 5 to 10 years. On average, the patients received 22 erythrocyte concentrates per year, to a mean individual total of 180 sediments. Each blood transfusion was accompanied by an intravenous infusion of deferoxamine 2 grams. Systematic blood perfusions unquestionably had beneficial effects on the frequency of sickling crises and complications, as well as on the patients' quality of life and psycho-social status. On the other hand, the iron overload could not be avoided, and it sometimes resulted in haematochromatosis (2 patients). At the moment, only continuous deferoxamine infusions could prevent this from happening and enable blood transfusions to be more widely used, provided its indications, restricted to the most severe forms of the disease, are carefully weighed. In this series of patients, the risk of viral contamination seemed to be relatively low, and in particular no HIV seroconversion was detected.
Laulan
S
S
Service d'Hématologie Clinique, Hôpital Beaujon, Clichy.
Bernard
J F
JF
Boivin
P
P
D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000097:blood / Q000235:genetics / Q000628:therapy*; D001803:Blood Transfusion; D017707:Erythrocyte Transfusion; D005260:Female; D005500:Follow-Up Studies; D006720:Homozygote; D006801:Humans; D007501:Iron / Q000097:blood; D008099:Liver / Q000098:blood supply; D008297:Male
D007501:Iron
Transfusions sanguines systématiques dans la drépanocytose homozygote de l'adulte. Etude de 11 cas suivis pendant 5 à 10 ans.