SECONDARY ALVEOLAR PROTEINOSIS IS A REVERSIBLE CAUSE OF RESPIRATORY-FAILURE IN LEUKEMIC PATIENTS
SECONDARY ALVEOLAR PROTEINOSIS IS A REVERSIBLE CAUSE OF RESPIRATORY-FAILURE IN LEUKEMIC PATIENTS
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DOI：
10.1164/ajrccm.149.3.8118651
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发表时间：
1994-03-01
影响因子：
24.7
通讯作者：
BERNAUDIN, JF
中科院分区：
文献类型：
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作者：
CORDONNIER, C;FLEURYFEITH, J;BERNAUDIN, JF
我们在此报告我们的经验，继发性肺泡蛋白沉积症（PAP）的血液恶性肿瘤患者。PAP的诊断是通过支气管肺泡灌洗（BAL），并根据鉴定的高碘酸希夫阳性蛋白质物质与特征性的超微结构模式。本文报告10例白血病继发PAP。三名患者接受了骨髓移植。从序贯BAL获得的数据表明，至少有四个人，他们都达到完全缓解或骨髓移植后中性粒细胞减少症的恢复，有可逆的PAP，我们强调这种潜在的可逆性。此外，为了估计PAP在血液病患者中的频率，我们回顾性研究了我科2年内发生的113例肺炎。在所有血液学人群中，继发性PAP在有肺部症状的患者中的发生率估计为5.3%，在髓系疾病患者中为10%。本报告（1）证实了BAL是诊断免疫功能低下患者PAP的准确方法，（2）强调PAP并非该人群呼吸衰竭的罕见原因，且与髓系疾病密切相关，（3）表明继发性PAP可能可逆，特别是在基础疾病完全缓解的情况下。
We report here our experience of secondary pulmonary alveolar proteinosis (PAP) in patients with hematologic malignancies. The diagnosis of PAP was made by bronchoalveolar lavage (BAL) and based on the identification of periodic acid-Schiff-positive proteinaceous material with the characteristic ultrastructural pattern. Ten patients with leukemia and secondary PAP are described. Three patients had received bone marrow transplants. Data obtained from sequential BAL have shown that at least four of them-all of them achieving complete remission or recovery from neutropenia after bone marrow transplantation-had reversible PAP, and we emphasize this potential reversibility. Furthermore, in order to estimate the frequency of PAP in hematologic patients, we retrospectively studied 113 episodes of pneumonia occurring in our department over a 2-yr period. The incidence of secondary PAP in patients with pulmonary symptoms was so estimated at 5.3% among all the hematologic population, and to 10% in patients with myeloid disorders. This report (1) confirms that BAL is an accurate way to diagnose PAP in immunocompromised hosts, (2) emphasizes that PAP is not an unusual cause of respiratory failure in this population and that it is strongly associated with myeloid disorders, and (3) shows that secondary PAP is potentially reversible, especially if complete remission of the underlying disease is achieved.