Case report: splenomegaly and splenic sequestration in an adult with sickle cell anemia

The American Journal of the Medical Sciences
S Moll, E P Orringer

Abstract

Splenomegaly in adult patients with homozygous sickle cell anemia (HbSS) is uncommon and splenic sequestration crises are rare. This paper describes a patient with HbSS who, at the age of 24, began to experience acute splenic sequestration crises. These episodes occurred with sufficient frequency and severity to warrant splenectomy. This case is presented to emphasize that, although rare, splenomegaly can persist in adults with homozygous HbSS and can be associated with severe and even life-threatening splenic sequestration. The incidence of splenomegaly in adults with HbSS and the factors linked to it will be discussed and the published reports of splenic sequestration crises in this patient population reviewed. It appears that high hemoglobin F (HbF) levels and alpha-thalassemia may be important etiologic factors in causing persistence of splenomegaly and predisposing patients to splenic sequestration crises.

References

Mar 1, 1979·Annals of Internal Medicine·O F Ballester, J Warth
Nov 1, 1970·British Journal of Haematology·G R Serjeant
Jun 17, 1982·The New England Journal of Medicine·D R HiggsG R Serjeant

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Citations

May 18, 2006·Annals of Hematology·Prasad Rao Koduri, Paula Kovarik
May 22, 2010·Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie·I DiagneH D Sow
Nov 29, 2011·Intensive Care Medicine·Nicolas de ProstArnaud W Thille
May 13, 2009·Journal of the American Academy of Nurse Practitioners·Ardie Pack-Mabien, Johnson Haynes
Mar 14, 2012·British Journal of Hospital Medicine·Aniket N TavareNichola Cooper

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