Painful crisis

Painful crisis characteristically causes severe pain and, occasionally, swelling of the juxta-articular areas of the long bones, sternum, ribs, thoracic and lumbar spine, and pelvis. These sites have in common the presence of active bone marrow and the common pathology is bone marrow necrosis, as evidenced by the aspiration of dead bone marrow from painful sites and the loss of normal activity on bone scans. The mechanism leading to avascular necrosis is controversial, and several lines of evidence conflict with the usual assumption of localized vaso-occlusion.

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