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Sickle Cell Disease
A. Carlson Merrow, Jr., MD, FAAP
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KEY FACTS

  • Imaging

    • Pathology

      • Clinical Issues

        • Diagnostic Checklist

          TERMINOLOGY

          • Abbreviations

            • Sickle cell disease (SCD); hemoglobin SS (Hb SS, Hgb SS)
          • Definitions

            • SCD: Hb SS (homozygous) → severe anemia
            • Sickle cell-hemoglobin C disease: Hb SC → mild anemia
            • Sickle cell-α thalassemia: Hb S-α thal → severe anemia
            • Sickle cell-β thalassemia: Hb S-β thal → mild to severe anemia
            • Sickle cell trait: Hb SA (heterozygous gene carrier)
            • Normal: Hb AA

          IMAGING

          • General Features

            • Radiographic Findings

              • MR Findings

                • Ultrasonographic Findings

                  • Nuclear Medicine Findings

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Microscopic Features

                          CLINICAL ISSUES

                          • Presentation

                            • Demographics

                              • Natural History & Prognosis

                                • Treatment

                                  DIAGNOSTIC CHECKLIST

                                  • Consider

                                    • Image Interpretation Pearls

                                      Selected References

                                      1. Kao CM et al: Microbiology and radiographic features of osteomyelitis in children and adolescents with sickle cell disease. Pediatr Blood Cancer. 67(10):e28517, 2020
                                      2. Fontalis A et al: The challenge of differentiating vaso-occlusive crises from osteomyelitis in children with sickle cell disease and bone pain: a 15-year retrospective review. J Child Orthop. 13(1):33-9, 2019
                                      3. Daltro G et al: Use of autologous bone marrow stem cell implantation for osteonecrosis of the knee in sickle cell disease: a preliminary report. BMC Musculoskelet Disord. 19(1):158, 2018
                                      4. Mesleh Shayeb A et al: Vaso-occlusive crisis as a predictor of symptomatic avascular necrosis in children with sickle cell disease. Pediatr Blood Cancer. 65(12):e27435, 2018
                                      5. Whitehead MT et al: Cerebral blood flow and marrow diffusion alterations in children with sickle cell anemia after bone marrow transplantation and transfusion. AJNR Am J Neuroradiol. 39(11):2132-9, 2018
                                      6. Kosaraju V et al: Imaging of musculoskeletal manifestations in sickle cell disease patients. Br J Radiol. 90(1073):20160130, 2017
                                      7. Sundu C et al: Bilateral subperiosteal hematoma and orbital compression syndrome in sickle cell disease. J Craniofac Surg. 28(8):e775-6, 2017
                                      8. Roberts AS et al: Extramedullary haematopoiesis: radiological imaging features. Clin Radiol. 71(9):807-14, 2016
                                      9. Delgado J et al: Utility of unenhanced fat-suppressed T1-weighted MRI in children with sickle cell disease -- can it differentiate bone infarcts from acute osteomyelitis? Pediatr Radiol. 45(13):1981-7, 2015
                                      10. Morrissey BJ et al: Incidence and predictors of bacterial infection in febrile children with sickle cell disease. Hemoglobin. 39(5):316-9, 2015
                                      11. Sachan AA et al: Is MRI necessary for skeletal evaluation in sickle cell disease. J Clin Diagn Res. 9(6):TC08-12, 2015
                                      12. Ganguly A et al: Musculoskeletal manifestations of sickle cell anaemia: a pictorial review. Anemia. 2011:794283, 2011
                                      13. Hernigou P et al: Septic arthritis in adults with sickle cell disease often is associated with osteomyelitis or osteonecrosis. Clin Orthop Relat Res. 468(6):1676-81, 2010
                                      14. Saito N et al: Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics. 30(4):1021-34, 2010
                                      15. Berger E et al: Sickle cell disease in children: differentiating osteomyelitis from vaso-occlusive crisis. Arch Pediatr Adolesc Med. 163(3):251-5, 2009
                                      16. Cerci SS et al: Different findings in Tc-99m MDP bone scintigraphy of patients with sickle cell disease: report of three cases. Ann Nucl Med. 21(5):311-4, 2007
                                      17. Ejindu VC et al: Musculoskeletal manifestations of sickle cell disease. Radiographics. 27(4):1005-21, 2007
                                      18. Almeida A et al: Bone involvement in sickle cell disease. Br J Haematol. 129(4):482-90, 2005
                                      19. 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
                                      20. Lonergan GJ et al: Sickle cell anemia. Radiographics. 21(4):971-94, 2001
                                      21. Naran AD et al: Sickle cell disease with orbital infarction and epidural hematoma. Pediatr Radiol. 31(4):257-9, 2001
                                      22. Rucknagel DL: The role of rib infarcts in the acute chest syndrome of sickle cell diseases. Pediatr Pathol Mol Med. 20(2):137-54, 2001
                                      23. 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
                                      24. 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
                                      25. Marlow TJ et al: "Tower vertebra": a new observation in sickle cell disease. Skeletal Radiol. 27(4):195-8, 1998
                                      26. Howlett DC et al: The role of CT and MR in imaging the complications of sickle cell disease. Clin Radiol. 52(11):821-9, 1997
                                      27. Gelfand MJ et al: Simultaneous occurrence of rib infarction and pulmonary infiltrates in sickle cell disease patients with acute chest syndrome. J Nucl Med. 34(4):614-8, 1993
                                      Related Anatomy
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                                      Related Differential Diagnoses
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                                      References
                                      Tables

                                      Tables

                                      KEY FACTS

                                      • Imaging

                                        • Pathology

                                          • Clinical Issues

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Sickle cell disease (SCD); hemoglobin SS (Hb SS, Hgb SS)
                                              • Definitions

                                                • SCD: Hb SS (homozygous) → severe anemia
                                                • Sickle cell-hemoglobin C disease: Hb SC → mild anemia
                                                • Sickle cell-α thalassemia: Hb S-α thal → severe anemia
                                                • Sickle cell-β thalassemia: Hb S-β thal → mild to severe anemia
                                                • Sickle cell trait: Hb SA (heterozygous gene carrier)
                                                • Normal: Hb AA

                                              IMAGING

                                              • General Features

                                                • Radiographic Findings

                                                  • MR Findings

                                                    • Ultrasonographic Findings

                                                      • Nuclear Medicine Findings

                                                        DIFFERENTIAL DIAGNOSIS

                                                          PATHOLOGY

                                                          • General Features

                                                            • Microscopic Features

                                                              CLINICAL ISSUES

                                                              • Presentation

                                                                • Demographics

                                                                  • Natural History & Prognosis

                                                                    • Treatment

                                                                      DIAGNOSTIC CHECKLIST

                                                                      • Consider

                                                                        • Image Interpretation Pearls

                                                                          Selected References

                                                                          1. Kao CM et al: Microbiology and radiographic features of osteomyelitis in children and adolescents with sickle cell disease. Pediatr Blood Cancer. 67(10):e28517, 2020
                                                                          2. Fontalis A et al: The challenge of differentiating vaso-occlusive crises from osteomyelitis in children with sickle cell disease and bone pain: a 15-year retrospective review. J Child Orthop. 13(1):33-9, 2019
                                                                          3. Daltro G et al: Use of autologous bone marrow stem cell implantation for osteonecrosis of the knee in sickle cell disease: a preliminary report. BMC Musculoskelet Disord. 19(1):158, 2018
                                                                          4. Mesleh Shayeb A et al: Vaso-occlusive crisis as a predictor of symptomatic avascular necrosis in children with sickle cell disease. Pediatr Blood Cancer. 65(12):e27435, 2018
                                                                          5. Whitehead MT et al: Cerebral blood flow and marrow diffusion alterations in children with sickle cell anemia after bone marrow transplantation and transfusion. AJNR Am J Neuroradiol. 39(11):2132-9, 2018
                                                                          6. Kosaraju V et al: Imaging of musculoskeletal manifestations in sickle cell disease patients. Br J Radiol. 90(1073):20160130, 2017
                                                                          7. Sundu C et al: Bilateral subperiosteal hematoma and orbital compression syndrome in sickle cell disease. J Craniofac Surg. 28(8):e775-6, 2017
                                                                          8. Roberts AS et al: Extramedullary haematopoiesis: radiological imaging features. Clin Radiol. 71(9):807-14, 2016
                                                                          9. Delgado J et al: Utility of unenhanced fat-suppressed T1-weighted MRI in children with sickle cell disease -- can it differentiate bone infarcts from acute osteomyelitis? Pediatr Radiol. 45(13):1981-7, 2015
                                                                          10. Morrissey BJ et al: Incidence and predictors of bacterial infection in febrile children with sickle cell disease. Hemoglobin. 39(5):316-9, 2015
                                                                          11. Sachan AA et al: Is MRI necessary for skeletal evaluation in sickle cell disease. J Clin Diagn Res. 9(6):TC08-12, 2015
                                                                          12. Ganguly A et al: Musculoskeletal manifestations of sickle cell anaemia: a pictorial review. Anemia. 2011:794283, 2011
                                                                          13. Hernigou P et al: Septic arthritis in adults with sickle cell disease often is associated with osteomyelitis or osteonecrosis. Clin Orthop Relat Res. 468(6):1676-81, 2010
                                                                          14. Saito N et al: Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics. 30(4):1021-34, 2010
                                                                          15. Berger E et al: Sickle cell disease in children: differentiating osteomyelitis from vaso-occlusive crisis. Arch Pediatr Adolesc Med. 163(3):251-5, 2009
                                                                          16. Cerci SS et al: Different findings in Tc-99m MDP bone scintigraphy of patients with sickle cell disease: report of three cases. Ann Nucl Med. 21(5):311-4, 2007
                                                                          17. Ejindu VC et al: Musculoskeletal manifestations of sickle cell disease. Radiographics. 27(4):1005-21, 2007
                                                                          18. Almeida A et al: Bone involvement in sickle cell disease. Br J Haematol. 129(4):482-90, 2005
                                                                          19. 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
                                                                          20. Lonergan GJ et al: Sickle cell anemia. Radiographics. 21(4):971-94, 2001
                                                                          21. Naran AD et al: Sickle cell disease with orbital infarction and epidural hematoma. Pediatr Radiol. 31(4):257-9, 2001
                                                                          22. Rucknagel DL: The role of rib infarcts in the acute chest syndrome of sickle cell diseases. Pediatr Pathol Mol Med. 20(2):137-54, 2001
                                                                          23. 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
                                                                          24. 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
                                                                          25. Marlow TJ et al: "Tower vertebra": a new observation in sickle cell disease. Skeletal Radiol. 27(4):195-8, 1998
                                                                          26. Howlett DC et al: The role of CT and MR in imaging the complications of sickle cell disease. Clin Radiol. 52(11):821-9, 1997
                                                                          27. Gelfand MJ et al: Simultaneous occurrence of rib infarction and pulmonary infiltrates in sickle cell disease patients with acute chest syndrome. J Nucl Med. 34(4):614-8, 1993