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