Lamour C, Bergeron A
Service de pneumologie, CHU de Poitiers, Poitiers cedex, France.
Rev Mal Respir. 2011 Jun;28(6):e18-27. doi: 10.1016/j.rmr.2009.04.001. Epub 2011 Jun 8.
Non-infectious pulmonary complications of myelodysplastic syndromes and chronic myeloproliferative disorders are not rare but remain little known to respiratory physicians who may be confronted with various clinical pictures corresponding to different pathophysiological causes.
The few data in the literature only relate to isolated cases or small series. The non-infectious pulmonary complications of myelodysplastic syndromes and chronic myeloproliferative disorders can be classified into several clinical entities: tumour syndrome, pulmonary fibrosis or diffuse infiltrating pneumonia, autoimmune reactions including vasculitis, Sweet syndrome, organizing pneumonia, pulmonary alveolar proteinosis, pleural effusion and pulmonary arterial hypertension. The diagnosis is provided by the histology and management of these complications depends on the underlying pathology.
Myelodysplastic syndromes and myeloproliferative disorders are entities which are becoming better characterized and understood. Better knowledge of the pathophysiological mechanisms involved in these complications should improve their diagnosis and their management, which still remains complex.
骨髓增生异常综合征和慢性骨髓增殖性疾病的非感染性肺部并发症并不罕见，但对于可能面对各种对应不同病理生理原因临床症状的呼吸科医生来说，仍鲜为人知。
文献中的少量数据仅涉及个别病例或小系列病例。骨髓增生异常综合征和慢性骨髓增殖性疾病的非感染性肺部并发症可分为几种临床类型：肿瘤综合征、肺纤维化或弥漫性浸润性肺炎、自身免疫反应，包括血管炎、Sweet综合征、机化性肺炎、肺泡蛋白沉积症、胸腔积液和肺动脉高压。这些并发症的诊断依靠组织学检查，其治疗取决于潜在的病理情况。
骨髓增生异常综合征和骨髓增殖性疾病正逐渐得到更好的特征描述和理解。对这些并发症所涉及病理生理机制的深入了解应能改善其诊断和治疗，而目前其治疗仍很复杂。
