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
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
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
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
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
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
Kosaraju V et al: Imaging of musculoskeletal manifestations in sickle cell disease patients. Br J Radiol. 90(1073):20160130, 2017
Sundu C et al: Bilateral subperiosteal hematoma and orbital compression syndrome in sickle cell disease. J Craniofac Surg. 28(8):e775-6, 2017
Roberts AS et al: Extramedullary haematopoiesis: radiological imaging features. Clin Radiol. 71(9):807-14, 2016
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
Morrissey BJ et al: Incidence and predictors of bacterial infection in febrile children with sickle cell disease. Hemoglobin. 39(5):316-9, 2015
Sachan AA et al: Is MRI necessary for skeletal evaluation in sickle cell disease. J Clin Diagn Res. 9(6):TC08-12, 2015
Ganguly A et al: Musculoskeletal manifestations of sickle cell anaemia: a pictorial review. Anemia. 2011:794283, 2011
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
Saito N et al: Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics. 30(4):1021-34, 2010
Berger E et al: Sickle cell disease in children: differentiating osteomyelitis from vaso-occlusive crisis. Arch Pediatr Adolesc Med. 163(3):251-5, 2009
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
Ejindu VC et al: Musculoskeletal manifestations of sickle cell disease. Radiographics. 27(4):1005-21, 2007
Almeida A et al: Bone involvement in sickle cell disease. Br J Haematol. 129(4):482-90, 2005
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
Lonergan GJ et al: Sickle cell anemia. Radiographics. 21(4):971-94, 2001
Naran AD et al: Sickle cell disease with orbital infarction and epidural hematoma. Pediatr Radiol. 31(4):257-9, 2001
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
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
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
Marlow TJ et al: "Tower vertebra": a new observation in sickle cell disease. Skeletal Radiol. 27(4):195-8, 1998
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
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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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
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
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
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
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
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
Kosaraju V et al: Imaging of musculoskeletal manifestations in sickle cell disease patients. Br J Radiol. 90(1073):20160130, 2017
Sundu C et al: Bilateral subperiosteal hematoma and orbital compression syndrome in sickle cell disease. J Craniofac Surg. 28(8):e775-6, 2017
Roberts AS et al: Extramedullary haematopoiesis: radiological imaging features. Clin Radiol. 71(9):807-14, 2016
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
Morrissey BJ et al: Incidence and predictors of bacterial infection in febrile children with sickle cell disease. Hemoglobin. 39(5):316-9, 2015
Sachan AA et al: Is MRI necessary for skeletal evaluation in sickle cell disease. J Clin Diagn Res. 9(6):TC08-12, 2015
Ganguly A et al: Musculoskeletal manifestations of sickle cell anaemia: a pictorial review. Anemia. 2011:794283, 2011
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
Saito N et al: Clinical and radiologic manifestations of sickle cell disease in the head and neck. Radiographics. 30(4):1021-34, 2010
Berger E et al: Sickle cell disease in children: differentiating osteomyelitis from vaso-occlusive crisis. Arch Pediatr Adolesc Med. 163(3):251-5, 2009
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
Ejindu VC et al: Musculoskeletal manifestations of sickle cell disease. Radiographics. 27(4):1005-21, 2007
Almeida A et al: Bone involvement in sickle cell disease. Br J Haematol. 129(4):482-90, 2005
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
Lonergan GJ et al: Sickle cell anemia. Radiographics. 21(4):971-94, 2001
Naran AD et al: Sickle cell disease with orbital infarction and epidural hematoma. Pediatr Radiol. 31(4):257-9, 2001
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
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
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
Marlow TJ et al: "Tower vertebra": a new observation in sickle cell disease. Skeletal Radiol. 27(4):195-8, 1998
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
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
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