Sickle cell anemia: a review of the imaging findings

Rosado, E; Paixão, P; Schmitt, W; Penha, D; Carvalho, F; Tavares, A

http://hdl.handle.net/10400.10/1168

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Sickle cell anemia - a review of the imaging findings LEARNING OBJECTIVES: To review and describe the manifestations of sickle cell anemia, focusing on the typical imaging findings in the most frequent affected organs. BACKGROUND: Sickle cell anemia is an autosomal recessive genetic condition characterized by a defective form of hemoglobin (hemoglobin S), which promotes the aggregation and distortion of red blood cells. Anemia results from the rapid removal of the abnormal red blood cells by the reticuloendothelial system. Another consequence of the presence of abnormal circulating cells is the obstruction of microcirculation, producing ischemia and infarction. Infarcts are common in several organs and they are responsible for most clinical and radiological manifestations. IMAGING FINDINGS: Sickle cell anemia can affect several organs. The lungs are frequently involved, with infarcts, emboli and increased propensity to pneumonia. Bone infarcts are also common causing acute pain crisis. Osteomyelitis is a frequent complication and its diagnosis can be challenging. In the brain, infarcts occur at young ages, causing cognitive impairment and neurologic deficits. Splenic infarcts progress over time to autosplenectomy. An important splenic complication is the sequestration syndrome, which may be life-threatening. Liver and kidneys may experience infarction as well. Pigmented biliary stones are also a very common finding due to hemolysis. CONCLUSION: In patients with sickle cell anemia, most relevant clinical and radiological manifestations are caused by ischemic complications. Lungs, bones, brain, kidneys, liver and spleen are among the most affected organs, with important clinical consequences. We have reviewed the imaging appearances of those lesions and correlated it to the physiopathology and clinical manifestations of the disease.