Gacouin A, Le Tulzo Y, Suprin E, Briens E, Bernard M, Camus C, Thomas R
Service de Réanimation Medicale, CHRU Rennes, Hôpital Pontchaillou, France.
Intensive Care Med. 1998 Mar;24(3):265-7. doi: 10.1007/s001340050563.
Pulmonary alvelolar proteinosis (PAP) is a rare cause of chronic respiratory failure due to progressive alveolar accumulation of a periodic acid-schiff (PAS) positive proteinaceous material. In some cases, the rapid accumulation of intra-alveolar material leads to acute respiratory failure (ARF). We report the causative role of secondary PAP in the case of a 26-year-old man with acute myeloid leukemia who developed fever, increased serum lactate dehydrogenase level and ARF, and required mechanical ventilation. The diagnosis of PAP was established by the examination of material obtained by bronchoalveolar lavage (BAL). Respiratory improvement occurred several days after the patient had recovered from neutropenia. This report underlines the importance of the early diagnosis of PAP as a potential cause of ARF in leukemic patients. Adequate stain on BAL fluid provides the diagnosis and avoids repeated invasive procedures and inappropriate treatments.
肺泡蛋白沉积症（PAP）是慢性呼吸衰竭的一种罕见病因，其由于周期性酸-希夫（PAS）阳性蛋白质物质在肺泡内进行性蓄积所致。在某些情况下，肺泡内物质的快速蓄积会导致急性呼吸衰竭（ARF）。我们报告了一名26岁急性髓系白血病男性患者继发PAP的致病作用，该患者出现发热、血清乳酸脱氢酶水平升高及ARF，并需要机械通气。通过对支气管肺泡灌洗（BAL）获取的物质进行检查确诊为PAP。患者从中性粒细胞减少症恢复数天后呼吸状况改善。本报告强调了早期诊断PAP作为白血病患者ARF潜在病因的重要性。对BAL液进行充分染色可实现诊断，并避免重复进行侵入性操作和不恰当治疗。
