Death due to sickle cell anaemia, an autopsy diagnosis: a study at a tertiary care hospital.

ABSTRACT

Background: Sickle cell disease (SCD) is the generic term for the group of inherited haemoglobinopathies caused by the occurrence of Haemoglobin S (HbS) in the homozygous or heterozygous form in combination of Hbs with another abnormal haemoglobin such as HbSC or beta-thalassaemias (HbS-thal). Sickle cell syndromes are remarkable for their clinical heterogeneity, including their presentations as sudden and unexpected deaths due to a sickle cell crisis. Less numbers of deaths are reported due to this cause because of ignorance of autopsy surgeon in considering this disease as a cause of death despite of its high prevalence. While doing autopsy in cases of deaths with no apparent cause and physical over activity medical officer must keep in mind the possibility of death due to vaso-occlusive crisis in sickle cell disease. Methods: The study covers a period of one year (January 2013 - December 2013) and it is a study of cases of autopsy carried out in a tertiary care hospital of South Gujarat. Results: A total of 607 cases examined, out of which sickled red blood cells were detected in 17 cases. The respective records were reviewed. Out of 17 cases, 13 cases were male and 4 cases were females. The youngest person was 15 years female and oldest was 70 years male. Conclusion: Sickle cell crisis is one of the causes of sudden unexplained deaths. The present study highlights the role of autopsy in such cases. Community awareness and marriage counseling programs are also helpful in preventing sickle cell disease.