Severe sclerosis of the long bodies in sickle cell anemia.

Abstract

In reviewing the radiographs of 102 patients with known sickle cell anemia, a 7% incidence (seven patients) of severe long-bone sclerosis was found. Of the affected patients 50% had homozygous sickle cell (SS) disease and 50% had sickle cell-hemoglobin C (SC) or S-thalassemia disease. No distinguishing radiographic features were found between these two groups. Those patients with SS disease and long-bone sclerosis were younger, had lower hemoglobin levels, and many more hospital admissions than those with SC or S-thalassemia disease and long-bone sclerosis. A review of the medical charts of these osteosclerotic patients with SS disease showed a probable positive correlation between infarctive episodes and hospital admissions in these patients. Therefore, patients with SS disease and severe long-bone sclerosis are seen much more frequently by the radiologist than those with SC or S-thalassemia and long-bone changes. These observations help negate any impression that severe long-bone sclerosis occurs frequently in and has a predilection for patients with homozygous SS disease.