肺淋巴瘤样肉芽肿病研究进展

Progress in the Studies of Pulmonary Lymphomatoid Granulomatosis

肺淋巴瘤样肉芽肿病是一种罕见的肺部疾病,可累及多个器官。好发于免疫缺陷的患者,病因尚不十分清楚,目前多认为是与EB病毒感染相关的富含T细胞的B细胞淋巴瘤。临床表现为咳嗽、咳痰、胸痛及呼吸困难等;病理形态表现为显著的血管炎、多种炎细胞浸润及坏死。按EB病毒感染性不典型大细胞的数量多少组织学分为三级,不同分级对疾病的侵袭性、治疗与预后有所不同。本病结局是变化不定的,少数患者未经治疗即消退,但大多数患者预后不佳。

Lymphomatoid granulomatosis（LYG）is a rare pulmonary disease,and many organs can be involved.LYG more often occurs in patients who have immunodeficiency,with unclear causes so far,while the current popular view is it′s a B-cell lymphoma with abundant T cells correlated with Epstein Barr virus（EBV）infection.Patients with LYG present with cough,expectoration,chest pain and dyspnea.LYG shows significant vasculitis,inflammatory cells infiltration and necrosis in histopathology.There is a histologic grading system for LYG based on the number of atypical large EBV-infected cells,which influences the invasion,treatment and prognosis of LYG.Outcome is variable.LYG may resolve without treatment,but the most common result is not well.