Transfusion requirements and effects in patients with thalassaemia major. Cooleycare Programme

Analysis of data available in 1985 on 3468 Italian and Greek patients registered in Cooleycare, an international cooperative programme of quality assessment of treatment delivery in thalassaemia, gave the following picture of treatment requirements and effects. The proportion of patients undergoing splenectomy has progressively decreased, and age at splenectomy has increased with time over the past 20 years. Age at first transfusion exceeds 4 years in a small but important group of patients, which indicates that a milder form of thalassaemia exists in this group. Children receiving modern treatment remain of near-normal stature until age 11 but later tend to be stunted. The mean blood requirement is 35% higher in non-splenectomised than in splenectomised patients. Differences in transfusion interval of 2 to 4 weeks have no measurable effect on blood requirement. Mean blood requirement rises gradually with mean haemoglobin concentration, possibly in a non-linear fashion. The prevalence of red cell alloimmunisation rises with delay in start on transfusion. Transfusion reactions were reported in 1% of transfusions (90% of which were leucocyte-depleted), from 17% of patients.