title: Acute splenic sequestration crisis in adults with hemoglobin S-C disease: a report of nine cases.
abstract: Acute splenic sequestration crisis (ASSC) is a potentially life-threatening complication and one of the leading causes of death in children with sickle cell disease. It is rarely reported in adults with hemoglobin S-C disease and its natural history in these patients has not been well studied. We describe here the clinicopathological features of ASSC in nine adults with hemoglobin S-C disease treated between 1972 and 2000 and followed for a mean period of 9 years (range 0-21 years). ASSC was characterized by acute left upper quadrant abdominal pain, splenomegaly, fever, and a rapid decrease in hematocrit with active erythropoiesis. The hemoglobin decreased by a mean of 4.8 g/dl from the steady state value (range 3.0 to 6.7 g/dl) during ASSC. Two patients failed to respond to transfusion of packed erythrocytes and required urgent splenectomy. There was one fatality-a 76-year-old woman, who died 36 h after admission. There was no recurrence of ASSC in five patients followed for 2, 3, 16, 18, and 21 years, respectively. ASSC in adults is a serious and occasionally, fatal complication of hemoglobin S-C disease. Patients with hemoglobin S-C disease may remain at risk of ASSC into their eighth decade.
authors.affiliation: The Division of Hematology-Oncology, Cook County Hospital, Chicago, IL 60612, USA. prkoduri@yahoo.com
authors.forename: Prasad Rao
authors.initials: PR
authors.lastname: Koduri
authors.forename: Sunita
authors.initials: S
authors.lastname: Nathan
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D004920:Erythropoiesis; D017809:Fatal Outcome; D005260:Female; D005334:Fever / Q000209:etiology; D006450:Hemoglobin SC Disease / Q000150:complications* / Q000473:pathology / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D013156:Splenectomy; D013158:Splenic Diseases / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D013163:Splenomegaly / Q000209:etiology