Sickle Cell Trait: Is It Always Benign?

Abstract

Sickle cell disease is a well-known homozygous inherited hemoglobinopathy that causes vaso-occlusive phenomena and chronic hemolysis. Vaso-occlusion results in sickle cell crisis and can eventually lead to complications involving multiple organ systems. However, the heterozygous counterpart, sickle cell trait (SCT) has less clinical significance as these patients are generally asymptomatic. This case series examines three unrelated patients with SCT ranging from the age of 27 to 61 years, who presented with pain in multiple long bones. Hemoglobin electrophoresis confirmed a diagnosis of SCT. Radiographic images of the affected sites showed osteonecrosis (ON). Interventions included pain management and bilateral hip replacement in two of the patients. Historically, vaso-occlusive disease in patients with SCT with no evidence of hemolysis or other hallmark findings of sickle cell disease is rare. There are limited reported cases of ON in SCT patients. Clinicians should explore other hemoglobinopathies not tested on routine hemoglobin electrophoresis and alternative risk factors for ON in these patients.