Death due to microvascular occlusion in sickle-cell trait following physical exertion

The heterozygous condition characterized by the presence of hemoglobin AS ( sickle-cell trait) occurs in approximately 8% of the American black populat ion. Unlike the homozygous state (sickle-cell disease), sickle-cell trait i s not widely recognized as a cause of life-threatening illness or death des pite over 30 case reports describing fatal or serious complications of exer cise in young black males with this condition. These reports identify heat stress, dehydration, viral illness, and poor physical conditioning as facto rs which may contribute to exertional rhabdomyolysis and sudden death, sugg esting multifactorial etiology. However, since sickling is known to occur p ostmortem, it remains controversial as to whether the pathogenesis of these exercise related deaths involves microvascular obstruction by sickled eryt hrocytes. We describe three young black individuals with no significant pas t medical history who died following physical exertion. In all three cases, postmortem hemoglobin electrophoresis demonstrated hemoglobin AS. In none of the cases was the body temperature found to be elevated. These cases ser ve to remind the forensic community that, in the proper setting, sickle-cel l trait must be viewed as a potentially fatal disorder.