Persistent Lung Lesion in a 6-year old Boy with Sickle Cell Disease

摘要

A 6-year-old boy with sickle cell disease (hemoglobin SS), who is on a long-term exchange transfusion program for repeated splenic sequestration and abnormal transcranial Doppler findings, presents to the emergency department with upper respiratory symptoms and a sore throat. On physical examination he appears well. His temperature is 99.3°F (37.4°C), heart rate is 100 beats/min, respiratory rate is 18 breaths/min, blood pressure is 110/65 mm Hg, and oxygen saturation is 100% on room air. A chest radiograph shows a lesion in the left upper lobe (Fig 1), thought to be round pneumonia, and the child is started on cefepime and azithromycin. It is noted that a very similar lesion was seen on radiography at the time of a previous hospital admission for upper respiratory infection 6 months earlier. Due to persistence of the opacity, a chest computed tomographic scan is performed and reveals a well-circumscribed round mass measuring 4x4x4 cm in the left hilum, centered at the bifurcation of the upper and lower bronchi (Fig 2). The mass is solid, homogeneous, and without calcification. A left thoracotomy and complete excision of the mass is completed. The specimen is sent to the pathology laboratory, and this leads to the eventual diagnosis.