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Sickle Cell Disease
Umesh D. Oza, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Diagnostic Checklist

        TERMINOLOGY

        • Abbreviations

          • Sickle cell disease (SCD)
        • Definitions

          • Genetic disorder of hemoglobin
            • Single base substitution in gene encoding beta-globin subunit
            • Deformed (sickled) RBCs
            • Causes wide range of clinical manifestations (e.g., vasoocclusive crisis → bone infarction, autosplenectomy → increased risk of infection)
          • Acute musculoskeletal pain in SCD: Primary differential = bone infarction vs. osteomyelitis
            • Both present with pain, swelling, fever
            • Blood cultures can be negative in up to 50% of cases of acute osteomyelitis
            • Treatments vastly different
              • Bone infarction: Analgesics, hydration
              • Osteomyelitis: Long-term antibiotics
            • Early diagnosis important
            • Bone infarction more common than osteomyelitis

        IMAGING

        • General Features

          • Nuclear Medicine Findings

            • Radiographic Findings

              • MR Findings

                • Ultrasonographic Findings

                  • Imaging Recommendations

                    DIFFERENTIAL DIAGNOSIS

                      PATHOLOGY

                      • General Features

                        • Gross Pathologic & Surgical Features

                          • Microscopic Features

                            CLINICAL ISSUES

                            • Presentation

                              • Demographics

                                • Natural History & Prognosis

                                  • Treatment

                                    DIAGNOSTIC CHECKLIST

                                    • Consider

                                      • Image Interpretation Pearls

                                        Selected References

                                        1. Pratesi A et al: Sickle cell-related bone marrow complications: the utility of diffusion-weighted magnetic resonance imaging. J Pediatr Hematol Oncol. 35(4):329-30, 2013
                                        2. Saito N et al: Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics. 30(4):1021-34, 2010
                                        3. Ziakas PD et al: Bone marrow necrosis in sickle cell anaemia. Blood Transfus. 8(3):211, 2010
                                        4. 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
                                        5. Ejindu VC et al: Musculoskeletal manifestations of sickle cell disease. Radiographics. 27(4):1005-21, 2007
                                        6. Adams BK et al: Technetium-99m MDP bone scintigraphy shows multiple abnormalities in sickle cell disease. Arch Dis Child. 88(7):631, 2003
                                        7. 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
                                        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. Kim HC et al: Differentiation of bone and bone marrow infarcts from osteomyelitis in sickle cell disorders. Clin Nucl Med. 14(4):249-54, 1989
                                        Related Anatomy
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                                        Related Differential Diagnoses
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                                        References
                                        Tables

                                        Tables

                                        KEY FACTS

                                        • Terminology

                                          • Imaging

                                            • Diagnostic Checklist

                                              TERMINOLOGY

                                              • Abbreviations

                                                • Sickle cell disease (SCD)
                                              • Definitions

                                                • Genetic disorder of hemoglobin
                                                  • Single base substitution in gene encoding beta-globin subunit
                                                  • Deformed (sickled) RBCs
                                                  • Causes wide range of clinical manifestations (e.g., vasoocclusive crisis → bone infarction, autosplenectomy → increased risk of infection)
                                                • Acute musculoskeletal pain in SCD: Primary differential = bone infarction vs. osteomyelitis
                                                  • Both present with pain, swelling, fever
                                                  • Blood cultures can be negative in up to 50% of cases of acute osteomyelitis
                                                  • Treatments vastly different
                                                    • Bone infarction: Analgesics, hydration
                                                    • Osteomyelitis: Long-term antibiotics
                                                  • Early diagnosis important
                                                  • Bone infarction more common than osteomyelitis

                                              IMAGING

                                              • General Features

                                                • Nuclear Medicine Findings

                                                  • Radiographic Findings

                                                    • MR Findings

                                                      • Ultrasonographic Findings

                                                        • Imaging Recommendations

                                                          DIFFERENTIAL DIAGNOSIS

                                                            PATHOLOGY

                                                            • General Features

                                                              • Gross Pathologic & Surgical Features

                                                                • Microscopic Features

                                                                  CLINICAL ISSUES

                                                                  • Presentation

                                                                    • Demographics

                                                                      • Natural History & Prognosis

                                                                        • Treatment

                                                                          DIAGNOSTIC CHECKLIST

                                                                          • Consider

                                                                            • Image Interpretation Pearls

                                                                              Selected References

                                                                              1. Pratesi A et al: Sickle cell-related bone marrow complications: the utility of diffusion-weighted magnetic resonance imaging. J Pediatr Hematol Oncol. 35(4):329-30, 2013
                                                                              2. Saito N et al: Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics. 30(4):1021-34, 2010
                                                                              3. Ziakas PD et al: Bone marrow necrosis in sickle cell anaemia. Blood Transfus. 8(3):211, 2010
                                                                              4. 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
                                                                              5. Ejindu VC et al: Musculoskeletal manifestations of sickle cell disease. Radiographics. 27(4):1005-21, 2007
                                                                              6. Adams BK et al: Technetium-99m MDP bone scintigraphy shows multiple abnormalities in sickle cell disease. Arch Dis Child. 88(7):631, 2003
                                                                              7. 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
                                                                              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. Kim HC et al: Differentiation of bone and bone marrow infarcts from osteomyelitis in sickle cell disorders. Clin Nucl Med. 14(4):249-54, 1989