Radiologic Manifestations of Sickle Cell Hemoglobinopathy

Abstract

SICKLE CELL hemoglobinopathy is capable of producing distinctive radiological signs in many organ systems. In the homozygous state (SS), it is a relatively common disorder, being present at birth in one of 625 black Americans.¹Its victims suffer a debilitating, frequently painful illness. Symptomatic forms of this hemoglobinopathy also occur when a single S gene is inherited in combination with the gene for another abnormal hemoglobin such as β thalassemia (S Thal) or hemoglobin C (SC).²The clinical course and the frequency and magnitude of roentgenographic signs in patients with the mixed forms of sickle cell disease vary greatly among persons with these genotypes. In some, the condition is almost indistinguishable from SS, whereas others exhibit few if any of the stigmas of their hereditary disorder. While there are some notable exceptions, symptoms and roentgenographic findings are usually absent in persons with sickle cell trait (AS), since the