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Abstract：
Although pulmonary events are considered to be frequently associated with malignant haemopathies, they have been sparsely studied in the specific context of myelodysplastic syndromes (MDS). We aimed to describe their different types, their relative proportions and their relative effects on overall survival (OS). We conducted a multicentre retrospective cohort study. Patients with MDS (diagnosed according to the 2016 WHO classification) and pulmonary events were included. The inclusion period was 1 January 2007 to 31 December 2017 and patients were monitored until August 2019. Fifty-five hospitalized patients were included in the analysis. They had 113 separate pulmonary events. Thirteen patients (23.6%) had a systemic autoimmune disease associated with MDS. Median age at diagnosis of MDS was 77 years. Median time to onset of pulmonary events was 13 months. Pulmonary events comprised: 70 infectious diseases (62%); 27 interstitial lung diseases (23.9%), including 13 non-specific interstitial pneumonias and seven secondary organizing pneumonias or respiratory bronchiolitis-interstitial lung diseases; 10 pleural effusions (8.8%), including four cases of chronic organizing pleuritis with exudative effusion; and six pulmonary hypertensions (5.3%). The median OS of the cohort was 29 months after MDS diagnosis but OS was only 10 months after a pulmonary event. The OS was similar to that of the general myelodysplastic population. However, the occurrence of a pulmonary event appeared to be either an accelerating factor of death or an indicator for the worsening of the underlying MDS in our study. More than a third of pulmonary events were non-infectious and could be systemic manifestations of MDS.
摘要：
尽管肺事件被认为经常与恶性血液病相关，在骨髓增生异常综合征（MDS）的特定背景下对它们进行了稀疏研究。我们旨在描述它们的不同类型，它们的相对比例及其对总生存期（OS）的相对影响。我们进行了一项多中心回顾性队列研究。包括MDS患者（根据2016年WHO分类诊断）和肺事件。纳入期为2007年1月1日至2017年12月31日，监测患者至2019年8月。55例住院患者纳入分析。他们有113个单独的肺事件。13例（23.6％）患有与MDS相关的系统性自身免疫性疾病。诊断为MDS的中位年龄为77岁。肺事件发生的中位时间为13个月。肺事件包括：70种感染性疾病（62%）；27种间质性肺病（23.9%），包括13例非特异性间质性肺炎和7例继发性机化性肺炎或呼吸性毛细支气管炎-间质性肺病;10例胸腔积液(8.8%),包括4例慢性组织性胸膜炎伴渗出性积液；和6例肺动脉高压（5.3％）。该队列的中位OS为MDS诊断后29个月，但OS仅在肺部事件后10个月。OS与一般骨髓增生异常群体相似。然而,在我们的研究中,肺部事件的发生似乎是死亡的加速因素或潜在MDS恶化的指标.超过三分之一的肺部事件是非感染性的，可能是MDS的全身性表现。
