Acute splenic sequestration crisis is a rare disorder that usually occurs in children, with sickle cell anemia, who are under the age of five years. A few cases have been described in adults with heterozygous sickle cell syndromes. Though this entity can be fatal there have been no reported cases associated with sudden death. We describe a case of sudden, unexpected death, associated with splenic sequestration, in a 29-year-old African-American man with undiagnosed sickle cell-beta-thalassemia syndrome.
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