A review of the causes of death in 276 patients with sickle-cell disease showed that although the greatest mortality occurred in the first five years of life, roughly one-quarter were aged over 30. Commonest causes of death in the first ten years included acute splenic sequestration, septicaemia, meningitis, aplastic crises, and gastroenteritis. In older patients cerebrovascular accidents and renal failure became common. The acute chest syndrome affected all age groups about equally but appeared to result predominantly from infection in the young and embolism or thrombosis in the old. IntroductionMost genotypes of sickle-cell disease are associated with a shortened life expectancy, but there is little information on the causes of death or on the pattern of mortality at different ages. Our experience at a major medical centre in the Caribbean over the past 30 years is therefore presented in this retrospective review of the causes of death in 276 patients.

Pure red cell aplasia (PRCA) is a recognized but rare complication of systemic lupus erythematosus (SLE) and is characterized by the near absence of red blood cell precursors in the bone marrow but with normal megakaryocyte and granulocytes. We report a novel case of acquired PRCA occurring simultaneously with immune thrombocytopenia in the context of active SLE. Both syndromes were refractory to conventional treatment but responded to rituximab and cyclophosphamide.