Case Report

Acute Splenic Sequestration Crisis Resembling Sepsis in an Adult with Hemoglobin SC Disease

Authors: Andrea Wang-Gillam, MD, PHD, Rita Shi-Ming Lee, MD, Eric D. Hsi, MD, Daniel J. Brotman, MD

Abstract

Acute splenic sequestration crisis (ASSC) is a common complication of sickle cell anemia in children. ASSC is generally not seen in adults with the SS genotype but occasionally can be seen in adults with the SC genotype. We present a case of fulminant ASSC in an adult with hemoglobin SC who developed high fever, intense abdominal pain, leukocytosis, and jaundice.

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References

1. Orringer EP, Fowler VG Jr, Owens CM, et al. Case report: splenic infarction and acute splenic sequestration in adults with hemoglobin SC disease. Am J Med Sci 1991; 302: 374–379.
 
2. Koduri PR, Agbemadzo B, Nathan S. Hemoglobin SC disease revisited: clinical study of 106 adults. Am J Hematol 2001; 68: 298–300.
 
3. Solanki DL, Kletter GG, Castro O. Acute splenic sequestration crises in adults with sickle cell disease. Am J Med 1986; 80: 985–990.
 
4. Sears DA, Udden MM. Splenic infarction, splenic sequestration, and functional hyposplenism in hemoglobin SC disease. Am J Hematol 1985; 18: 261–268.
 
5. Shao SH, Orringer EP. Case report: splenic sequestration and multiorgan failure as the presenting manifestation of hemoglobin SC disease. Am J Med Sci 1996; 311: 139–141.
 
6. Embury S, Vichinsky E. Sickle cell disease, in Silberstein L, McGlave P (eds): Hematology: Basic Principles and Practice. New York, Churchill Livingstone, 2000, pp 523–524.
 
7. Bannister L. Haemolymphoid system, in Bannister L (ed): Gray’s Anatomy. New York, Churchill Livingstone, 1995, ed 38, pp 1399–1442.
 
8. Aquino VM, Norvell JM, Buchanan GR. Acute splenic complications in children with sickle cell-hemoglobin C disease. J Pediatr 1997; 130: 961–965.
 
9. Michel JB, Hernandez JA, Buchanan GR. A fatal case of acute splenic sequestration in a 53-year-old woman with sickle-hemoglobin C disease. Am J Med 1992; 92: 97–100.
 
10. Grover R, Wethers DL. Management of acute splenic sequestration crisis in sickle cell disease. J Assoc Acad Minor Phys 1990; 1: 67–70.
 
11. Sheth S, Ruzal-Shapiro C, Piomelli S, et al. CT imaging of splenic sequestration in sickle cell disease. Pediatr Radiol 2000; 30: 830–833.