Hypersplenism in Sickle Cell Anemia

Although splenomegaly in children with sickle cell anemia is common, massive enlargement associated with thrombocytopenia and very severe anemia is rare. In such patients, splenectomy has been followed by increase in platelet count and hemoglobin concentration. This suggests that an acquired hypersplenism aggravated the hemolytic process. The present report concerns a 6-year-old boy with sickle cell anemia demonstrating this unusual association. Studies were done in an effort to define the nature of the acquired extracorpuscular defect (hypersplenism). We also observed improvement after splenectomy as previously noted by others.^1-6

Report of Case  The patient is a 6-year-old Negro male born Oct 18, 1957. He was admitted to Milwaukee County General Hospital for the first time in February, 1960. At that time the hemoglobin concentration was 5.9 gm%, and the peripheral smear revealed microcytosis and marked hypochromia. Iron therapy was begun and by March, 1960, the hemoglobin had risen to 7.0