1. Systemic Pulmonary Events Associated with Myelodysplastic Syndromes: A Retrospective Multicentre Study
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Anne-Laure Lejeune, Louis Terriou, D. Launay, Lidwine Stervinou-Wemeau, Quentin Scanvion, Arsène Mekinian, Laurent Pascal, Valérie Deken, Bruno Quesnel, Eric Hachulla, Thierno Sy, CHU Lille, CNRS, Inserm, Université de Lille, Université Lille Nord (France), METRICS : Evaluation des technologies de santé et des pratiques médicales - ULR 2694, Institut de Recherche Translationnelle sur l'Inflammation (INFINITE) - U1286, Hôpital Saint Vincent de Paul de Lille, Cancer Heterogeneity, Plasticity and Resistance to Therapies (CANTHER) - UMR 9020 - UMR 1277, Service de Pneumologie et Immuno-Allergologie [CHU LIlle], Centre Hospitalier Régional Universitaire [Lille] [CHRU Lille], Miniaturisation pour la Synthèse, l'Analyse et la Protéomique (MSAP) - USR 3290, and CHU Saint-Antoine [AP-HP]
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medicine.medical_specialty ,pulmonary alveolar proteinosis ,Population ,lcsh:Medicine ,Context (language use) ,[SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract ,Article ,03 medical and health sciences ,0302 clinical medicine ,Internal medicine ,pulmonary hypertension ,medicine ,pneumonia ,10. No inequality ,education ,030304 developmental biology ,interstitial lung disease ,0303 health sciences ,education.field_of_study ,Lung ,business.industry ,lcsh:R ,Interstitial lung disease ,Retrospective cohort study ,[SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology ,General Medicine ,medicine.disease ,Pulmonary hypertension ,3. Good health ,medicine.anatomical_structure ,030220 oncology & carcinogenesis ,Cohort ,iatrogenic effects ,pleuritic effusion ,business ,Pulmonary alveolar proteinosis - 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.
- Published
- 2021
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