Sickle cell trait as a contributory cause of death in natural disease

Sickle cell trait (SCT) affects 300 million people globally, and awareness is growing that SCT is not an entirely benign condition; however, most reported cases have been non-natural deaths. Autopsy records from the Baylor University Medical Center (BUMC) in Dallas, Texas, contained seven natural deaths from January 2007 to October 2013 in which micro-occlusive sickling was identified at autopsy and SCT confirmed by postmortem hemoglobin fractionation. Sickle crisis was never diagnosed clinically. These cases illustrate the importance of red cell morphology in autopsy material. When sickling is suspected, hemoglobin fractionation should be performed. If confirmed, SCT should be listed as an autopsy finding and the severity and distribution of sickling documented. Extensive micro-occlusive sickling should be considered contributory to death; however, its relative importance depends on all facts of the case. Accurate reporting should facilitate further research and the development of evidence-based preventative and supportive strategies for these patients.