Division of Pulmonary and Sleep Medicine, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Division of Critical Care Medicine, Department of Anesthesia and Critical Care, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
Pediatr Pulmonol. 2022 Jan;57(1):308-310. doi: 10.1002/ppul.25718. Epub 2021 Oct 13.
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.
肺泡蛋白沉积症（PAP）是指表面活性物质在肺泡腔中蓄积。继发性 PAP 已在多种疾病中报道，在极少数情况下与血液系统恶性肿瘤相关。PAP 的治疗基于潜在的疾病过程，可能包括全肺灌洗、吸入或皮下粒细胞-巨噬细胞集落刺激因子或他汀类药物。继发性 PAP 报道称对全肺灌洗的反应不佳。我们报告了一例使用全肺灌洗成功治疗急性髓系白血病合并继发性 PAP 的儿科患者。
