经尸体解剖病理证实的肺淋巴瘤样肉芽肿一例并文献复习

A case report of pulmonary lymphomatoid granulomatosis confirmed by autopsy and review of the literature

目的探讨肺淋巴瘤样肉芽肿(PLG)的临床、影像学特征、诊断、治疗及预后。方法报道1例经尸体解剖病理证实的PLG病例,并结合相关文献进行复习总结。结果患者男性,44岁,以咳嗽、咯痰、呼吸困难为主要临床表现。肺部CT影像学表现为双肺散在以血管为中心的结节影伴斑片影,下肺多见。临床抗感染及对症治疗无缓解。患者病情逐渐加重并死亡。尸体解剖显示为PLG,病理分级Ⅲ级,镜下以血管为中心的各种淋巴细胞浸润伴坏死,EBER原位杂交阳性。文献复习检索到中文文献28篇、外文文献34篇。文献总结PLG好发于中年男性,其发生与EBV感染有关,多有免疫功能低下,临床症状不典型,影像学主要表现为沿支气管血管束分布的多发结节及斑片影。确诊依靠病理组织学及免疫组织化学、EBER原位杂交,治疗局部病变以手术为主,进展较快的Ⅰ、Ⅱ级患者和所有Ⅲ级患者,常用RCHOP方案进行联合化疗。其预后与分级相关,Ⅲ级侵袭性强、预后差。结论PLG是一种临床表现不典型的原发于肺的淋巴增生性疾病,见于多种免疫抑制性疾病。其CT影像学有一定的特征性,但确诊需结合病理。部分患者经治疗可缓解,Ⅲ级患者预后差。

Objective To investigate the clinical,imaging features,diagnosis,treatment and prognosis of pulmonary lymphomatoid granulomatosis(PLG).Methods A case of PLG confirmed by autopsy and pathology was reported,and the related literature was reviewed.Results A 44-year-old male patient presented with cough,expectoration and shortness of breath as the main symptoms.The imaging findings of lung CT were diffuse vascular nodular and patchy changes in the lungs,especially in the lower lung.Clinical symptoms were not relieved with antiinfection and symptomatic treatment.The patient’s condition gradually exacerbated and finally died.The autopsy revealed PLG with pathological gradeⅢ.The infiltration of lymphocytes centered with blood vessel and necrosis were observed under light microscope.EBER in situ hybridization was positive.Totally 28 Chinese articles and 34 foreign articles were screened out.Literature review suggested that PLG usually occurred in middle-aged men and was associated with EBV infection.It is frequent with immunodepression.Its clinical symptoms were atypical.The main imaging manifestations were multiple nodules and masses more frequent along the bronchovascular bundle.A diagnosis of PLG depended on pathological histology,immunohistochemistry and EBER in situ hybridization.Surgery was the main treatment for local lesions.The patients of grade I andⅡwith rapid progress and all gradeⅢwere usually treated with RCHOP regimen combined with chemotherapy.The prognosis was correlated with grading.GradeⅢwas aggressive and the prognosis was poor.Conclusions PLG is an atypical pulmonary lymphoproliferative disorder.It can be seen in many immunosuppressive diseases.The CT imaging features of PLG have certain characteristics,but the final diagnosis should be combined with pathological diagnosis.Some patients can be relieved by treatment,and patients with gradeⅢhave poor prognosis.