Introduction: Sickle Cell Disease (SCD) has a high mortality rate in the environment where we practice. There is lack of contemporal autopsy studies describing causes of death among SCD patients at our centre.

Methods: This is a retrospective study of SCD patients who died between January 1991 and December 2008 and that had autopsy examination to confirm the cause of death in a Nigerian teaching hospital. The clinical data, including the age, gender, Hb genotype, and the major autopsy findings and cause of death were obtained for each patient from the complete autopsy reports that included histopathological examination. Multiple causes of death were entertained.

Results: A total of 52 autopsies were performed. The mean age at death was 21.3 years (range, 1-47 years) and a male/female ratio of 1.3:1. HbS+C patients lived longer than HbS patients (21.0 years Vs 24.0 years) and peak mortality was in the 2nd and 3rd decades of life. The commonest causes of death as a single entity or in combination included infections in 78% of cases, fatal thrombotic/embolic events (37%) making acute chest syndrome a leading cause of death. This was followed closely by anemia alone or in combination with acute sequestration crises in 31% of patients.

Conclusion: Infections are the commonest causes of death in Nigerian SCD patients, efforts to reduce infection especially early in life through prophylaxis or vaccination will impact on the overall survival of these patients.