Pathogenesis of Lung Disease in Sickle Hemoglobinopathies

Pulmonary complications are major contributors to acute morbidity in patients with sickle cell disease (SCD), and in the long run, lung involvement constitutes a paramount determinant of survival. Most patient care and research has been directed toward general hematologic aspects of SCD with the result that pulmonary aspects of this disorder remain poorly understood. In part this reflects the fact that pulmonary consequences of SCD have received little attention by clinicians and investigators interested in the lung and pulmonary circulation. This workshop was convened to promote exchange of ideas among hematologists experienced in problems presented by patients with SCD and physicians and investigators with background in lung and pulmonary vascular disorders. The intent was to summarize current knowledge concerning the pulmonary syndromes of SCD and to develop ideas about what should be done clinically and investigatively to promote better understanding and treatment in this area.