AMC Li

Clinical records of 129 transfusion-dependent β-thalassaemia major patients treated in this department over a thirty-four years period (1961 January 1st to 1994 December 31st) were reviewed with the aim to find out if patients have improved survivals. Eight patients who defaulted the follow up were excluded. According to the year they were born, patients were grouped into six 5-years periods, and one 4-years period (group 1-6, 1961-1990; group 7, 1991-1994). Survival figures for the seven groups are 2/18 (11.1%), 3/20 (15%), 18/25 (72%), 26/ 27 (96.3%), 16/19 (84.2%), 14/14 (100%), and 6/6 (100%) respectively. Altogether forty four patients had died. Causes of death were heart failure in 22 patients, infections in 11, liver failure in 2, refusal of treatment in 2, malignant tumours in 2, complication of bone marrow transplant in 1, and other causes in 4. There were no deaths in group 6 and 7. Even though the patient follow up period varies for the seven groups (from 1+ to 33+ years), the improved survival in patients born after 1970 is obvious (15% or less survived for group 1, 2, and greater than 70% from group 3 onward). The introduction of subcutaneous desferrioxamine as an effective iron chlation therapy in 1979 is an important contributing factor.