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Sickle Cell Disease
Kevin R. Moore, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              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

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        Selected References

                                        1. De Luna G et al: High bone mineral density in sickle cell disease: prevalence and characteristics. Bone. 110:199-203, 2018
                                        2. Eid R et al: Spinal cord infarction in hemoglobin SC disease as an amusement park accident. Pediatrics. 138(3), 2016
                                        3. Nix JS et al: Spinal bone marrow necrosis with vertebral compression fracture: differentiation of BMN from AVN. Skeletal Radiol. 43(9):1337-40, 2014
                                        4. 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
                                        5. 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
                                        6. Porter JB: Pathophysiology of transfusional iron overload: contrasting patterns in thalassemia major and sickle cell disease. Hemoglobin. 33 Suppl 1:S37-45, 2009
                                        7. Sarrai M et al: Bone mass density in adults with sickle cell disease. Br J Haematol. 136(4):666-72, 2007
                                        8. 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
                                        9. Lonergan GJ et al: Sickle cell anemia. Radiographics. 21(4):971-94, 2001
                                        10. 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
                                        11. States LJ: Imaging of metabolic bone disease and marrow disorders in children. Radiol Clin North Am. 39(4):749-72, 2001
                                        12. 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
                                        13. Roger E et al: Sickle cell disease of the spine in children. Can J Surg. 42(4):289-92, 1999
                                        14. Marlow TJ et al: "Tower vertebra": a new observation in sickle cell disease. Skeletal Radiol. 27(4):195-8, 1998
                                        15. 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

                                        • Terminology

                                          • Imaging

                                            • Top Differential Diagnoses

                                              • Pathology

                                                • Clinical Issues

                                                  • Diagnostic Checklist

                                                    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

                                                                            DIAGNOSTIC CHECKLIST

                                                                            • Consider

                                                                              Selected References

                                                                              1. De Luna G et al: High bone mineral density in sickle cell disease: prevalence and characteristics. Bone. 110:199-203, 2018
                                                                              2. Eid R et al: Spinal cord infarction in hemoglobin SC disease as an amusement park accident. Pediatrics. 138(3), 2016
                                                                              3. Nix JS et al: Spinal bone marrow necrosis with vertebral compression fracture: differentiation of BMN from AVN. Skeletal Radiol. 43(9):1337-40, 2014
                                                                              4. 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
                                                                              5. 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
                                                                              6. Porter JB: Pathophysiology of transfusional iron overload: contrasting patterns in thalassemia major and sickle cell disease. Hemoglobin. 33 Suppl 1:S37-45, 2009
                                                                              7. Sarrai M et al: Bone mass density in adults with sickle cell disease. Br J Haematol. 136(4):666-72, 2007
                                                                              8. 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
                                                                              9. Lonergan GJ et al: Sickle cell anemia. Radiographics. 21(4):971-94, 2001
                                                                              10. 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
                                                                              11. States LJ: Imaging of metabolic bone disease and marrow disorders in children. Radiol Clin North Am. 39(4):749-72, 2001
                                                                              12. 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
                                                                              13. Roger E et al: Sickle cell disease of the spine in children. Can J Surg. 42(4):289-92, 1999
                                                                              14. Marlow TJ et al: "Tower vertebra": a new observation in sickle cell disease. Skeletal Radiol. 27(4):195-8, 1998
                                                                              15. 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