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 anti-infection and symptomatic treatment. The patient's condition gradually exacerbated and finally died. The autopsy revealed PLG with pathological grade Ⅲ. 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 and Ⅱ with rapid progress and all grade Ⅲ were usually treated with RCHOP regimen combined with chemotherapy. The prognosis was correlated with grading. Grade Ⅲ was 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 grade Ⅲ have poor prognosis.

Citation： ZHANG Youyi, LI Juan. A case report of pulmonary lymphomatoid granulomatosis confirmed by autopsy and review of the literature. Chinese Journal of Respiratory and Critical Care Medicine, 2019, 18(6): 567-572. doi: 10.7507/1671-6205.201811036 Copy