[Monitoring and appraisal of the effects of blood transfusion and iron chelation in thalassemia major].
A total of 21 patients with thalassaemia major between 2 and 20-years-old were on a "hypertransfusion" regimen for 1 to 14 year periods. The use of the transfusion quotient (TQ) allowed a precise survey of the blood requirement. In all splenectomized patients the TQ was between 1 and 2; TQ increased and was always greater than 2 when patients became hypersplenic. Of the 21 regularly transfused patients, 12 had iron chelation therapy by continuous subcutaneous desferrioxamine (DF) injection (1 g over 8 to 12 h at home, nightly). The iron balance was negative for 7 patients, equilibrated for 1 and positive for 4 patients. All patients receiving more than injections of DF weekly had a negative balance; the variation of the serum ferritin level correlated with the iron balance (r = 0,77; P less than 0,01). The beneficial effects of the treatment were improvement in social and professional activity, in growth and puberty development and attenuation of the skull and face dysmorphia. Monitoring ferritin is a simple way to estimating the effects of chelation on iron overload.
D000293:Adolescent; D000328:Adult; D001787:Blood Group Incompatibility / Q000209:etiology; D002648:Child; D002657:Child Development / Q000502:physiology; D002675:Child, Preschool; D003676:Deferoxamine / Q000009:adverse effects; D005260:Female; D005293:Ferritins / Q000097:blood; D006509:Hepatitis B / Q000209:etiology; D006801:Humans; D007502:Iron Chelating Agents / Q000009:adverse effects*; D008297:Male; D013156:Splenectomy; D013789:Thalassemia / Q000097:blood / Q000503:physiopathology / Q000628:therapy*; D065227:Transfusion Reaction
D007502:Iron Chelating Agents; D005293:Ferritins; D003676:Deferoxamine
Surveillance et estimation des effets de la transfusion sanguine et de la chélation du fer dans la thalassémie majeure.