Hemoglobin SC disease presenting as acute chest syndrome with ventilation-perfusion mismatches

Acute chest syndrome, characterized by fever, chest pain and pulmonary infiltrates, is a known complication of hemoglobin SC disease. A 41-year-old African male with previously unknown hemoglobinopathy developed an acute episode of fever, chest pain and dyspnea right after surgery for retinal detachment. The patient was suspected of having pulmonary thromboembolism. This was further suggested by a 'high probability' ventilation-perfusion scan. However, a pulmonary angiogram revealed no evidence of thromboembolism. Subsequently, the patient was recognized to have hemoglobin SC disease, with the acute chest syndrome. After appropriate treatment with antibiotics, hydration and exchange transfusion, the patient underwent another surgery without complications.