Hemoglobin S-C disease presenting as acute pneumonitis with pulmonary angiographic findings in two patients

Abstract

Acute illness characterized by fever, cough, chest pain and pulmonary infiltrates on chest film Is characteristic of patients with sickle cell anemia and with sickle-C disease. The underlying hemoglobinopathy is usually recognized in the former, but because patients with sickle-C disease as a rule have less severe anemia and fewer, less severe crises their abnormal hemoglobin may not be detected until adolescence or adulthood. Acute pulmonary illness in such patients may therefore present a diagnostic dilemma for the unwary. Two cases are presented to highlight this point. Pulmonary angiographic findings in patients with sickle cell states might add ionformation about the pathogenesis of this disorder since vascular occlusion appears to play a major role. Pulmonary anglograms in the two patients we describe documented the presence of localized abnormalities of perfusion. In one, the presence of filling defects in medium-sized arteries suggests intravascular thromboembolism.