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Findings In all patients the bone has abnormal architecture on the radiographs with some patients having focal areas of sclerosis and others with more diffuse sclerosis. On the MRI, the bone is again abnormal in all patients with geographic areas representing infarcts and then other more diffuse abnormal signal which is slightly too hyperintense on the fat suppressed images and often too low in signal on the PD and especially T1 images. The vertebrae also show a characteristic central depression or H shaped pattern. In all patients the bone has abnormal architecture on the radiographs with some patients having focal areas of sclerosis and others with more diffuse sclerosis. On the MRI, the bone is again abnormal in all patients with geographic areas representing infarcts and then other more diffuse abnormal signal which is slightly too hyperintense on the fat suppressed images and often too low in signal on the PD and especially T1 images. The vertebrae also show a characteristic central depression or H shaped pattern.
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Diagnosis: Sickle Cell Disease A full discussion of the pathology of sickle cell disease is beyond the scope of this presentation. Suffice to say it is related to abnormal hemoglobin that causes a sickling of the red blood cells and precipitates infarcts of various end organs. This is seen in the bone as well as the spleen. In addition, there is an increased degree of erythropoietic marrow to help combat the anemia of sickle cell disease and hence the diffuse abnormal signal on MRI. The more diffuse sclerosis is thought to relate to either multiple infarcts of the bone or end stage fibrosis/mineralization. The breakdown of the red cells also produces a lot of bilirubin that can form gallstones and often necessitates cholecystectomy. There is an increased risk of thromboembolic disease in these patients that is multifactorial but often necessitates placement of an IVC filter particularly before an orthopedic procedure. Related often to the infarction of the spleen as well as decrease marrow available for production of white blood cells, these patients are at a great risk to develop infections of all sorts.
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References Musculoskeletal Manifestations of Sickle Cell Disease. Vivian C. Ejindu, MRCP, Andrew L. Hine, FRCR, Mohammad Mashayekhi, FRCR, Philip J. Shorvon, FRCR, and Rakesh R. Misra, FRCR. Radiographics. 2007 July August. Sickle Cell Anemia. Gael J. Lonergan, Lt Col, USAF, MC, David B. Cline, MAJ, MC, USA, and Susan L. Abbondanzo, MD. Radiographics. 2001 July.
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