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

Acute chest syndrome, characterized by fever, chest pain and pulmonary infi ltrates, is a known complication of hemoglobin SC disease. A 41-year-old Af rican male with previously unknown hemoglobinopathy developed an acute epis ode of fever, chest pain a nd dyspnea right after surgery for retinal detac hment. The patient was suspected of having pulmonary thromboembolism. This was further suggested by a 'high probability' ventilation-perfusion scan. H owever, a pulmonary angiogram revealed no evidence of thromboembolism. Subs equently, the patient was recognized to have hemoglobin SC disease, with th e acute chest syndrome. After appropriate treatment with antibiotics, hydra tion and exchange transfusion, the patient underwent another surgery withou t complications.