Successful whole lung lavage in a child with pulmonary alveolar proteinosis secondary to hematologic malignancy

Abstract

Pulmonary alveolar proteinosis (PAP) describes the accumulation of surfactant in the alveolar space. Secondary PAP has been reported in a variety of diseases, and in rare cases has been associated with hematologic malignancy. Treatment for PAP is based on the underlying disease process, and may include whole lung lavage, inhaled or subcutaneous granulocyte‐macrophage colony‐stimulating factor, or statins. PAP secondary to hematologic malignancy has been reported to demonstrate poor response to whole lung lavage. We report a case of successful treatment of a pediatric patient with acute myeloid leukemia and secondary PAP using whole lung lavage.

Alveolar macrophages in pulmonary alveolar proteinosis: origin, function, and therapeutic strategies

“…Therefore, restoring normal AM function to maintain pulmonary lipid and immune homeostasis is a fundamental approach for the treatment of primary PAP ( 108 ). In the treatment of secondary PAP, the primary approach is to address the underlying primary disease or remove the causative factors, with adjunctive whole lung lavage (WLL) being an additional option to enhance therapeutic efficacy in severe cases ( 109 , 110 ). Furthermore, gene therapy-induced restoration of normal AM function also serves as a therapeutic option for hereditary PAP ( 111 ).…”