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Sickle Cell Disease
Bryson Borg, MD
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KEY FACTS

  • Imaging

    • Top Differential Diagnoses

      • Pathology

        • Clinical Issues

          TERMINOLOGY

          • Synonyms

            • Sickle cell anemia, HbSS disease
          • Definitions

            • Hereditary hemoglobinopathy resulting in anemia and red blood cells that deform (sickle) and occlude blood vessels
            • Homozygous: HbSS (sickle cell anemia)
            • Heterozygous: HbSA (sickle cell trait, asymptomatic), HbSC (less severe form)
            • Sickle cell crisis: Acute episode of severe bone, abdomen, chest pain

          IMAGING

          • General Features

            • Radiographic Findings

              • CT Findings

                • MR Findings

                  • Imaging Recommendations

                    • Nuclear Medicine Findings

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  Selected References

                                  1. Nix JS et al: Spinal bone marrow necrosis with vertebral compression fracture: differentiation of BMN from AVN. Skeletal Radiol. 43(9):1337-40, 2014
                                  2. Das D et al: MRI appearances of extramedullary haematopoiesis presenting with cauda equina syndrome in sickle cell disease. Clin Radiol. 66(12):1219-22, 2011
                                  3. Gupta R et al: Pattern of bone mineral density in sickle cell disease patients with the high-Hb F phenotype. Acta Haematol. 123(1):64-70, 2010
                                  4. Porter JB: Pathophysiology of transfusional iron overload: contrasting patterns in thalassemia major and sickle cell disease. Hemoglobin. 33 Suppl 1:S37-45, 2009
                                  5. Sarrai M et al: Bone mass density in adults with sickle cell disease. Br J Haematol. 136(4):666-72, 2007
                                  6. Kim SK et al: Natural history and distribution of bone and bone marrow infarction in sickle hemoglobinopathies. J Nucl Med. 43(7):896-900, 2002
                                  7. Lonergan GJ et al: Sickle cell anemia. Radiographics. 21(4):971-94, 2001
                                  8. Skaggs DL et al: Differentiation between bone infarction and acute osteomyelitis in children with sickle-cell disease with use of sequential radionuclide bone-marrow and bone scans. J Bone Joint Surg Am. 83-A(12):1810-3, 2001
                                  9. States LJ: Imaging of metabolic bone disease and marrow disorders in children. Radiol Clin North Am. 39(4):749-72, 2001
                                  10. Umans H et al: The diagnostic role of gadolinium enhanced MRI in distinguishing between acute medullary bone infarct and osteomyelitis. Magn Reson Imaging. 18(3):255-62, 2000
                                  11. Roger E et al: Sickle cell disease of the spine in children. Can J Surg. 42(4):289-92, 1999
                                  12. Marlow TJ et al: "Tower vertebra": a new observation in sickle cell disease. Skeletal Radiol. 27(4):195-8, 1998
                                  13. Kahn CE Jr et al: Combined bone marrow and gallium imaging. Differentiation of osteomyelitis and infarction in sickle hemoglobinopathy. Clin Nucl Med. 13(6):443-9, 1988
                                  Related Anatomy
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                                  Related Differential Diagnoses
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                                  References
                                  Tables

                                  Tables

                                  KEY FACTS

                                  • Imaging

                                    • Top Differential Diagnoses

                                      • Pathology

                                        • Clinical Issues

                                          TERMINOLOGY

                                          • Synonyms

                                            • Sickle cell anemia, HbSS disease
                                          • Definitions

                                            • Hereditary hemoglobinopathy resulting in anemia and red blood cells that deform (sickle) and occlude blood vessels
                                            • Homozygous: HbSS (sickle cell anemia)
                                            • Heterozygous: HbSA (sickle cell trait, asymptomatic), HbSC (less severe form)
                                            • Sickle cell crisis: Acute episode of severe bone, abdomen, chest pain

                                          IMAGING

                                          • General Features

                                            • Radiographic Findings

                                              • CT Findings

                                                • MR Findings

                                                  • Imaging Recommendations

                                                    • Nuclear Medicine Findings

                                                      DIFFERENTIAL DIAGNOSIS

                                                        PATHOLOGY

                                                        • General Features

                                                          CLINICAL ISSUES

                                                          • Presentation

                                                            • Demographics

                                                              • Natural History & Prognosis

                                                                • Treatment

                                                                  Selected References

                                                                  1. Nix JS et al: Spinal bone marrow necrosis with vertebral compression fracture: differentiation of BMN from AVN. Skeletal Radiol. 43(9):1337-40, 2014
                                                                  2. Das D et al: MRI appearances of extramedullary haematopoiesis presenting with cauda equina syndrome in sickle cell disease. Clin Radiol. 66(12):1219-22, 2011
                                                                  3. Gupta R et al: Pattern of bone mineral density in sickle cell disease patients with the high-Hb F phenotype. Acta Haematol. 123(1):64-70, 2010
                                                                  4. Porter JB: Pathophysiology of transfusional iron overload: contrasting patterns in thalassemia major and sickle cell disease. Hemoglobin. 33 Suppl 1:S37-45, 2009
                                                                  5. Sarrai M et al: Bone mass density in adults with sickle cell disease. Br J Haematol. 136(4):666-72, 2007
                                                                  6. Kim SK et al: Natural history and distribution of bone and bone marrow infarction in sickle hemoglobinopathies. J Nucl Med. 43(7):896-900, 2002
                                                                  7. Lonergan GJ et al: Sickle cell anemia. Radiographics. 21(4):971-94, 2001
                                                                  8. Skaggs DL et al: Differentiation between bone infarction and acute osteomyelitis in children with sickle-cell disease with use of sequential radionuclide bone-marrow and bone scans. J Bone Joint Surg Am. 83-A(12):1810-3, 2001
                                                                  9. States LJ: Imaging of metabolic bone disease and marrow disorders in children. Radiol Clin North Am. 39(4):749-72, 2001
                                                                  10. Umans H et al: The diagnostic role of gadolinium enhanced MRI in distinguishing between acute medullary bone infarct and osteomyelitis. Magn Reson Imaging. 18(3):255-62, 2000
                                                                  11. Roger E et al: Sickle cell disease of the spine in children. Can J Surg. 42(4):289-92, 1999
                                                                  12. Marlow TJ et al: "Tower vertebra": a new observation in sickle cell disease. Skeletal Radiol. 27(4):195-8, 1998
                                                                  13. Kahn CE Jr et al: Combined bone marrow and gallium imaging. Differentiation of osteomyelitis and infarction in sickle hemoglobinopathy. Clin Nucl Med. 13(6):443-9, 1988