Incidence of AIDS in HIV-1 infected thalassaemia patients. European and Mediterranean W.H.O. Working Group on Haemoglobinopathies and Cooleycare.

To estimate the cumulative incidence of acquired immunodeficiency syndrome (AIDS) in thalassaemia major patients (TMP) human immunodeficiency virus (HIV-1) infected through transfusion, 79 seropositive TMP were studied. At inclusion, mean age was 12.4 +/- 6.6 years; 40 were men; 21 were splenectomized. Centers for Disease Control, 1986 (CDC) stages and prescription of zidovudine were noted at least once a year. Cumulative incidence of AIDS and standard error were calculated using non parametric life table method. Age, sex, acute infection and splenectomy associations with progression to AIDS were tested using Breslow statistic. The median follow-up period was 4 years 11 months. At the end of the study period, 43 TMP were in CDC stage II, 23 in CDC stage III and 13 in CDC stage IV, including seven AIDS cases, of whom three had died. Four subjects died of other causes. Only two patients were treated with AZT prior to the occurrence of AIDS. Rate of progression to AIDS was not associated with acute infection, splenectomy, age, or sex. A cumulative AIDS incidence rate of 1.4% (SE 1.3%) was observed at 3 years and of 9% (SE 4%) at 5 years.