Case Report of Acute Splenic Sequestration Crisis in an Adult Patient with Hb S Disease and Suspected Hereditary Persistence of Fetal Hemoglobin. (2nd January 2021)

• Record Type: Journal Article
• Authors: Sigal, Ian R.; Ciunci, Christine A.
• Is Part Of: Hemoglobin. Volume 45:Number 1(2021)
• Page Start: 60
• Page End: 61
• Publication Date: 2021-01-02
• Subjects: Acute splenic sequestration crisis (ASSC) -- hereditary persistence of fetal hemoglobin (HPFH) -- sickle cell anemia; Hemoglobinopathy -- Periodicals; Hemoglobin -- Periodicals; Hematology -- Periodicals; Thalassemia -- Periodicals; Blood -- Diseases -- Periodicals; 612.1111
• DOI: 10.1080/03630269.2020.1868495
• Languages: English
• ISSNs: 0363-0269

Abstract

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.