تظاهرات اسکلتی- عضلانی پیشرفته در یک بیمار مسن مبتلا به بیماری سلول داسی شکل، یک مورد نادر

Sickle cell disease is the most common type of hemoglobinopathies in the world that is caused by abnormal beta globin chain in hemoglobin. The disease is usually diagnosed in the first decade of life. Bone involvement is one of the most common clinical manifestations both in the acute setting (painful vaso-occlusive crises), and/or as a source of chronic disability (such as avascular necrosis). This paper introduces an old patient with advanced skeletal manifestation due to this disorder. A 66-year-old man who was opium abuser was visited by a rheumatologist for lower limbs pain, and fatigue from one year ago. The patient had severe anemia, and X-ray revealed diffuse osteolytic changes without sharp border in pelvis and femur and joint space narrowing. Involvement of thoracolumbar vertebras were seen as codfish vertebra appearance. In peripheral blood smear, sickle cell and target cells accompanied by hypochromia and platelets aggregation were detected. The hemoglobin electrophoresis was compatible with sickle-β+ thalassemia. In old patients with anemia and osteolytic lesions with unknown reason, evaluation of hemoglobinopathies, especially sickle cell anemia is suggested.