Sickle cell anaemia in a 60 year old Nigerian

The case of a sixty-year-old Nigerian with sickle cell anaemia is presented. His steady state haematocrit is 0.26 L/L. Haemoglobin F. and HbA2 measured 7.00% and 2.9% respectively. Bone pain crisis occurred very infrequently (one or less per year) but jaundice is always present. A right nephrectomy for haematuria was carried out in Dublin, Ireland, in 1954 when he had his first ever blood transfusion. He was hospitalised for the first time in Nigeria on 21/7/83 in anaemic cardiac failure with haematocrit of 0.14 L/L during which he had the second blood transfusion. Chronic leg ulcer, avascular necrosis of the femoral head and cholelithiasis were absent. He had led a fairly active life and recently retired (1986) as a bursar from a secondary school.