Pulmonary alveolar proteinosis treated with whole-lung lavage utilizing extracorporeal membrane oxygenation: a case report and review of literatures

摘要

Pulmonary alveolar proteinosis (PAP) was first described by Rosen et al in 1958. PAP is characterized by the accumulation of proteinaceous material in the alveoli leading to varying degrees of impairment in gas exchange. Recent studies demonstrate that deficiency of granulocyte-macrophage colony stimulating factor ( GM-CSF) is a strongly suspected mechanism for adult acquired PAP, and subcutaneous administration of recombinant human GM-CSF is effective in approximately 50% of patients with PAP; but whole lung lavage ( WLL) remains the most effective treatment. However, WLL may cause transient worsening of hypoxaemia, making the procedure hazardous in those patients most needing lavage. This technique is difficult and dangerous to perform in the most severely hypoxemic patients. We used venoarterial extracorporeal membrane oxygenation ( ECMO) for a patient with PAP, thus avoiding dangerous hypoxaemia during lavage. Up to now, few authors report having used the same approach for PAP.