Benefits and complications of regular blood transfusion in patients with beta-thalassaemia major.
Early and regular blood transfusion therapy in patients with homozygous beta-thalassaemia decreases the complications of severe anaemia and prolongs survival. In the long term, however, the beneficial effects of transfusions are limited by the organ damage resulting from iron overload, a consequence of the body's limited capacity to excrete iron, and by the complications of infection with blood-borne agents. Transfusion regimens for beta-thalassaemia have changed substantially during the past four decades. In current protocols, pre-transfusion haemoglobin concentration should not exceed 95 g/l. This allows adequate control of anaemia, with a relatively low rate of iron accumulation. Although iron chelation therapy has successfully improved survival free from cardiac disease, thalassaemic patients continuously present new clinical challenges. In fact, the vast majority of them suffer from post-transfusion chronic hepatitis C, which is expected to significantly contribute to morbidity in the forthcoming years. Furthermore, recent studies demonstrated that thalassaemics are at high risk of acquiring several blood-borne viruses. The potential role of these multiple infections in inducing clinical disease is still uncertain, and needs to be thoroughly clarified in future surveys.
Prati
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Centro Trasfusionale e di Immunologia dei Trapianti, IRCCS Ospedale Maggiore di Milano, Italia. dprati@yahoo.com
D000743:Anemia, Hemolytic / Q000209:etiology; D001787:Blood Group Incompatibility / Q000150:complications; D001803:Blood Transfusion; D002318:Cardiovascular Diseases / Q000209:etiology / Q000517:prevention & control; D002614:Chelating Agents / Q000627:therapeutic use; D015913:Chelation Therapy; D002986:Clinical Trials as Topic; D003676:Deferoxamine / Q000627:therapeutic use; D004198:Disease Susceptibility; D004700:Endocrine System Diseases / Q000209:etiology / Q000517:prevention & control; D015658:HIV Infections / Q000635:transmission; D006525:Hepatitis, Viral, Human / Q000517:prevention & control / Q000635:transmission; D006801:Humans; D007114:Immunization; D016867:Immunocompromised Host; D007501:Iron; D019190:Iron Overload / Q000209:etiology / Q000517:prevention & control; D007518:Isoantibodies / Q000096:biosynthesis; D008107:Liver Diseases / Q000209:etiology / Q000517:prevention & control; D065227:Transfusion Reaction; D017086:beta-Thalassemia / Q000150:complications / Q000628:therapy*
D002614:Chelating Agents; D007518:Isoantibodies; D007501:Iron; D003676:Deferoxamine