Persistent Spleen Enlargement in Sickle Cell Disease: An Unresolved Dilemma

Abstract

Sickle cell disease (SCD) is a hereditary blood disorder which affects mostly blacks of African descent. Spleen enlargement or splenomegaly is a common clinical manifestation in SCD, and normally develops progressively from about the age of 6 months, and is due mainly to repeated capturing and hemolysis of sickled red blood cells. The spleen then undergoes atrophy by the age of 6 years, as a result of vasoocclusion, ischemia and scarring. However, in a few cases, this splenomegaly may persist beyond 6 years, and may be responsible for frequent hemolytic episodes and acute splenic sequestration of red blood cells which can be fatal. The cause, role, consequences and management of a persistent spleen enlargement remains unclear, and is still an unresolved dilemma. This review discusses these various aspects in light of recent research on this issue. From this we conclude that, there is a wide array of clinical implications and therapeutic options, which have to be considered in the management of these patients to avert deadly complications.