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April 30, 1973

Section 49.—Sickle Cell Disease and Related Hemoglobinopathies

JAMA. 1973;224(Suppl_5):772. doi:10.1001/jama.1973.03220190112052

The crises of sickle cell disease are often associated with intense polyarthralgia, a finding which has been responsible for the mistaken diagnosis of rheumatic fever.1 On occasion the pain is accompanied by hydrarthroses and other evidence of joint inflammation, which may be the result of small synovial infarctions.2 The skeletal changes which are so characteristic a feature of this disease may be separated into those that are related to hyperplasia of the marrow and those that are a consequence of local sickle cell thrombosis and infarction.3 Included among the former are widening of medullary cavities, thinning of cortices, coarsening and irregularity of trabecular markings, and cupping of vertebral bodies. Most notable among the bone lesions believed to result from sickle cell thrombosis is avascular necrosis of the head of the femur and, less commonly, the head of the humerus, patella, and vertebral bodies (Fig 79).4 This complication has been