肺淋巴管平滑肌瘤病1例并文献复习

发布时间：2017-12-27 03:13

本文关键词：肺淋巴管平滑肌瘤病1例并文献复习　出处：《中医药临床杂志》2017年04期 　论文类型：期刊论文

【摘要】：目的:探讨肺淋巴管平滑肌瘤病(PLAM)的病理与临床表现、影像学特征、诊断和治疗,提高对该病的认识,减少误诊及漏诊。方法:回顾性分析1例我院临床确诊的PLAM患者病历资料,并复习相关文献。结果:本病主要发生于育龄期妇女,亦可发生于绝经后,本例PLAM患者发病于绝经后1年。PLAM肺部表现有活动后呼吸困难、咯血、反复发作的性气胸、乳糜胸等,肺外表现有乳糜腹、肾血管平滑肌脂瘤、子宫肌瘤、颅内病变等。HRCT的典型表现为双肺弥漫分布的薄壁囊状影。肺功能以阻塞性或混合性通气功能障碍为主。病理检查为确诊金标准。黑色素瘤相关的HMB45抗体绝大多数为阳性。血管内皮生长因子D(VEGF-D)有很高的敏感性和特异性,诊断该病有潜在价值。结论:目前本病病因尚不清,针对TSC1/TSC2基因突变的靶向药物雷帕霉素能改善部分患者症状及肺功能,具有一定疗效。
[Abstract]:Objective: To investigate the pathological and clinical manifestations, imaging features, diagnosis and treatment of pulmonary lymphatic leiomyomias (PLAM), and to improve the understanding of the disease and reduce misdiagnosis and missed diagnosis. Methods: a retrospective analysis of 1 cases of clinical records of PLAM patients in our hospital was reviewed, and the related literature was reviewed. Results: this disease mainly occurred in women of childbearing age, and could also occur after menopause. This case of PLAM patients occurred 1 years after menopause. PLAM manifestations of exertional dyspnea, hemoptysis, recurrent pneumothorax, chylothorax, lung manifestations of chyloperitoneum, renal vascular smooth muscle lipoma, uterine fibroids, intracranial lesions. The typical manifestation of HRCT is thin-walled cystic shadow in the diffuse distribution of two lungs. Pulmonary function is mainly obstructed or mixed ventilation dysfunction. The pathological examination was the standard of the diagnosis of gold. Most of the HMB45 antibodies associated with melanoma were positive. Vascular endothelial growth factor D (VEGF-D) has a high sensitivity and specificity, and it is of potential value in the diagnosis of this disease. Conclusion: the etiology of this disease is unclear, the mutation of TSC1/TSC2 gene targeted drug rapamycin can improve symptoms and pulmonary function, have certain curative effect.
【作者单位】：安徽中医药大学;安徽省中医院;
【基金】：国家中医药管理局“十二五”重点学科-中医老年病资助项目(NO 2012-170)
【分类号】：R734.2
【正文快照】： Pulmonary Lymphangiomyomatosis(One Case and Review of the Literature)HUANG He1,GONG Qiang-jing2,ZHANG Nian-zhi21.Anhui University of traditional Chinese Medicine,230038,China2.Anhui Chinese Medicine Hospital,230031,ChinaOne case of Pulmonary Lymphangiomy

【相似文献】