A fatal case of acute splenic sequestration in a 53-year-old woman with sickle-hemoglobin C disease.

Acute splenic sequestration crisis (ASSC) is a common complication in infants and young children with homozygous sickle cell disease, but it is infrequent in patients with sickle-hemoglobin C (SC) disease. When it does occur in such patients, it is often associated with exposure to high altitude, either by air travel or mountainous environment. Since 1970, only 15 cases of ASSC have been reported in patients with SC disease who were not exposed to a high altitude. Nine of these were children and adolescents aged 11 to 18 years, while six were adults aged 21 to 44 years. A review of these cases shows only two fatalities from ASSC: a 12-year-old West Indian girl living in England and a 13-year-old black girl in the United States. In this report, we describe the sudden death from ASSC of a 53-year-old black woman with SC disease at low altitude. To our knowledge, death from ASSC has not been previously reported in an adult patient with SC disease.