Case Report of Acute Splenic Sequestration Crisis in an Adult Patient with Hb S Disease and Suspected Hereditary Persistence of Fetal Hemoglobin

Abstract

Acute splenic sequestration crisis (ASSC) is a life-threatening complication of sickle cell disease characterized by acute splenic enlargement and a rapid drop in hemoglobin (Hb) levels. Although ASSC is predominantly a disease of childhood, rare cases of ASSC have been reported in adults, frequently in association with heterozygous sickle cell disease. We describe a case of ASSC in a 29-year-old woman with homozygous Hb S (HBB: c.20A>T) disease and suspected hereditary persistence of fetal Hb (HPFH), which was accompanied by marked leukocytosis.

Keywords:

• Acute splenic sequestration crisis (ASSC)
• hereditary persistence of fetal hemoglobin (HPFH)
• sickle cell anemia

Disclosure statement

The authors report no conflicts of interest. The authors alone are responsible for the content and writing of this article.