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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-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. 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
                                      2. Morrissey BJ et al: Incidence and Predictors of Bacterial infection in Febrile Children with Sickle Cell Disease. Hemoglobin. 39(5):316-9, 2015
                                      3. Sachan AA et al: Is MRI Necessary for Skeletal Evaluation in Sickle Cell Disease. J Clin Diagn Res. 9(6):TC08-12, 2015
                                      4. Ganguly A et al: Musculoskeletal manifestations of sickle cell anaemia: a pictorial review. Anemia. 2011:794283, 2011
                                      5. 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
                                      6. Saito N et al: Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics. 30(4):1021-34, 2010
                                      7. Berger E et al: Sickle cell disease in children: differentiating osteomyelitis from vaso-occlusive crisis. Arch Pediatr Adolesc Med. 163(3):251-5, 2009
                                      8. 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
                                      9. Ejindu VC et al: Musculoskeletal manifestations of sickle cell disease. Radiographics. 27(4):1005-21, 2007
                                      10. Almeida A et al: Bone involvement in sickle cell disease. Br J Haematol. 129(4):482-90, 2005
                                      11. 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
                                      12. Lonergan GJ et al: Sickle cell anemia. Radiographics. 21(4):971-94, 2001
                                      13. Naran AD et al: Sickle cell disease with orbital infarction and epidural hematoma. Pediatr Radiol. 31(4):257-9, 2001
                                      14. 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
                                      15. 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
                                      16. 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
                                      17. Marlow TJ et al: "Tower vertebra": a new observation in sickle cell disease. Skeletal Radiol. 27(4):195-8, 1998
                                      18. 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
                                      19. 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-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. 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
                                                                          2. Morrissey BJ et al: Incidence and Predictors of Bacterial infection in Febrile Children with Sickle Cell Disease. Hemoglobin. 39(5):316-9, 2015
                                                                          3. Sachan AA et al: Is MRI Necessary for Skeletal Evaluation in Sickle Cell Disease. J Clin Diagn Res. 9(6):TC08-12, 2015
                                                                          4. Ganguly A et al: Musculoskeletal manifestations of sickle cell anaemia: a pictorial review. Anemia. 2011:794283, 2011
                                                                          5. 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
                                                                          6. Saito N et al: Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics. 30(4):1021-34, 2010
                                                                          7. Berger E et al: Sickle cell disease in children: differentiating osteomyelitis from vaso-occlusive crisis. Arch Pediatr Adolesc Med. 163(3):251-5, 2009
                                                                          8. 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
                                                                          9. Ejindu VC et al: Musculoskeletal manifestations of sickle cell disease. Radiographics. 27(4):1005-21, 2007
                                                                          10. Almeida A et al: Bone involvement in sickle cell disease. Br J Haematol. 129(4):482-90, 2005
                                                                          11. 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
                                                                          12. Lonergan GJ et al: Sickle cell anemia. Radiographics. 21(4):971-94, 2001
                                                                          13. Naran AD et al: Sickle cell disease with orbital infarction and epidural hematoma. Pediatr Radiol. 31(4):257-9, 2001
                                                                          14. 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
                                                                          15. 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
                                                                          16. 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
                                                                          17. Marlow TJ et al: "Tower vertebra": a new observation in sickle cell disease. Skeletal Radiol. 27(4):195-8, 1998
                                                                          18. 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
                                                                          19. 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