CASE-REPORT - SPLENOMEGALY AND SPLENIC SEQUESTRATION IN AN ADULT WITHSICKLE-CELL-ANEMIA

Splenomegaly in adult patients with homozygous sickle cell anemia (HbS S) is uncommon and splenic sequestration crises are rare. This paper d escribes a patient with HbSS who, at the age of 24, began to experienc e acute splenic sequestration crises. These episodes occurred with suf ficient frequency and severity to warrant splenectomy. This case is pr esented 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 an d the published reports of splenic sequestration crises in this patien t population reviewed. It appears that high hemoglobin F (HbF) levels and alpha-thalassemia may be important etiologic factors in causing pe rsistence of splenomegaly and predisposing patients to splenic sequest ration crises.