Sickle Cell Disease

Abstract

The specimen was received from a 13-year-old girl with Salmonella heidelberg bacteremia. A magnetic resonance imaging (MRI) revealed septic arthritis of the left shoulder and possible osteomyelitis of the left femur. After a month of therapy with antibiotics, she developed a large soft tissue abscesses over her left hip. An area identified by MRI as a small focus of osteomyelitis was curetted. The curettage specimen showed fragments of bone and bone marrow with slightly decreased but maturing hematopoiesis. There were areas with plasma cells and neutrophils associated with focal destruction of the bony spicules; these findings indicate acute and chronic osteomyelitis. In addition, there are numerous drepanocytes featuring elongated, tapered ends, characteristic of sickle cell disease (Figure 1). These findings were then correlated with a clinical history of hemoglobin SS and a sickle cell crisis in this patient. However, and especially in the absence of a similar clinical history, it is important to communicate to the clinician the presence of drepanocytes in tissue sections in order to intervene and stave off numerous potentially severe manifestations of the disease, including vaso-occlusion, acute splenic sequestration, and aplastic or hemolytic crises. Bone and joint disease, in particular septic arthritis of the hip, is a frequent complication in sickle cell anemia. The major differential diagnosis in evaluating a sickle cell patient with bone pain includes osteomyelitis and bone infarction. Because clinical presentations are similar, and imaging is of limited value, the histological diagnosis is paramount.