肺淋巴管平滑肌瘤病的研究进展

New Concepts in Knowledge of Pulmonary Lymphangioleiomyomatosis

肺淋巴管平滑肌瘤病(PLAM)是一种由平滑肌细胞增生侵犯肺实质和肺淋巴管引起的缓慢进展性肺部疾病,与结节性硬化症基因突变有关。肺部损害表现为广泛的组织重塑,导致结构扭曲;可在胸、腹部合并存在淋巴管肌瘤或血管肌脂瘤。2000年前后,关于PLAM方面的研究和认识进展显著,并且很快应用到了实际的治疗中。就现有情况看,PLAM为一种单基因、温和的肿瘤生长方式,其分类既不是良性又不属于恶性。得益于其分子生物学基础方面的深入研究,靶向治疗有了长足的发展。肺移植是目前唯一有效的治疗方法,但移植后的肺组织中很可能出现淋巴管平滑肌细胞,形成疾病复发。

Pulmonary Lymphangioleiomyomatosis （PLAM） is a slowly progressive lung disease that is associated with mutations in tuberous sclerosis complex genes,infiltration of the pulmonary parenchyma and lymphatics with neoplastic smooth muscle cells,extensive tissue remodeling and architectural distortion of the lung,which may have the coexistence of the tumors in chest and abdomen,including lymphangiomyomas and angiomyolipemas.Around 2000,there has been remarkable progress in the understanding of PLAM and rapid translation of this knowledge to an effective therapy.PLAM is a mild,monogenic model of neoplasia,d neither benign nor malignant.Thanks to the deeper digging in molecular basis of PLAM,great progress has been made in the targeted therapy.However,the only proven treatment for PLAM is lung transplantation,though PLAM cells may probably return to the transplanted lung forming recurrence.