Sickle Cell Hemoglobinopathy And Sudden Death (pp. 125-130)
Authors: de la Grandmaison, G. Lorin (University R. Descartes, Paris;Raymond Poincaré Hospital, France)
Abstract: Although rare, sudden death is the most serious complication of sickle cell trait. Sickle cell trait has a substantially increased age-dependent risk of exercice-related sudden death. Sudden death may occur in susceptible persons when poor physical conditioning, dehydration, heat stresses or hypoxic states precipitate sickling of the abnormal erythrocytes. Most of the death mechanisms are related to the biological consequences of diffuse microvascular occlusion due to sickling, although a significant number of such sudden deaths remain unexplained after thorough autopsy. Rare mechanisms encountered include acute splenic sequestration. The main pathological findings include widespread intravascular sickling, but the exact cause of death is not always explained after a complete autopsy. Morphological findings are only indicative of the possible mechanism of death and do not prove it. A complete autopsy with systematic histologic examination, hemoglobin electrophoresis, vitreous electrolytes, urine myoglobin, microbiological studies and DNA analysis should be evaluated in any unexplained sudden death in a young African-American patient.