Sickle Cell Anemia (Homozygous S) with Aseptic Necrosis of Femoral Head

SINCE the advent of the newer technics for differentiating hemoglobin types,1–4 no case of sickle cell anemia (homozygous S) proven by these methods has been described in which aseptic necrosis of the femoral head was present. Aseptic necrosis of the femoral capital epiphysis has been reported by Smith and Conley5 to occur frequently in the genetic variants of sickle cell anemia. In this report, the complete absence of this lesion in their patients with sickle cell anemia was stressed. Several cases showing femoral head lesions and presumed to represent true sickle cell anemia have been reported,6,16 but these were not verified electrophoretically, nor were the clinical and hematologic data sufficient to make a definite clinical diagnosis of sickle cell anemia as distinct from its genetic variants. It is our purpose to present six patients with sickle cell anemia (homozygous S) proven by paper electrophoresis who demonstrated aseptic necrosis of the femoral head.