Functional Asplenia in Sickle-Cell Anemia

Abstract

Observation of Howell—Jolly bodies in the blood of children with sickle-cell anemia suggested the paradox of splenic hypofunction despite clinical splenomegaly. Spleen scans were performed with a sulfur colloid tagged with radioactive technetium (99mTc). No splenic uptake of the colloid was seen in 11 of 12 children with sickle-cell anemia, although many had enlarged spleens. Twenty-five controls, including children with sickle-cell trait and Hgb SC and Hgb S-β thalassemia diseases, regularly demonstrated splenic uptake. Functional asplenia in children with sickle-cell anemia may be a pertinent factor in the susceptibility to infections — particularly pneumococcal infections — that is characteristic of this disease.