Autopsy findings and pattern of mortality in undiagnosed sickle cell disease patients
Keywords:Sickle cell disease, Sudden and unexpected death, Vaso-occlusive crisis, Autopsy
Background: Sickle cell disease (SCD) has a high mortality rate with unexpected sudden death. So, the purpose of the study was to analyze clinical and/or autopsy findings at the time of death among sickle cell disease (SCD) patients.
Methods: This is a retrospective study of SCD patients who died between January 2011 to December 2011 and the morphological evidence of the cause of death was studied in a tertiary care hospital of south Gujarat. The clinical data, including the age, gender, symptoms and the major autopsy findings and cause of death were obtained for each patient that included histopathological examination.
Results: A total of 472 autopsies were performed, out of which sickled erythrocytes were detected in 40 cases. The mean age at death was 30 years and a male/female ratio of 3:1 and peak mortality was in the 2nd and 3rd decades of life. The common causes of death in this study include vaso-occlusive crisis (45%), infection (40%). The terminal infection was heralded by upper respiratory tract (43.7%) and by gastroenteritis (31.2%). Other causes of death included intracranial haemorrhage (2.5%) and cirrhosis (2.5%). Among the cases of SCD, 10% of deaths were non-haemoglobinopathy related such as fall (accidental), organophosphorus poisoning and snake bite.
Conclusion: This study is to precise analysis of causes of death is needed to focus and improve morbidity and mortality in sickle cell disease especially in highly prevalent area and will impact on the overall survival of these patients.
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