Pulmonary lymphomatoid granulomatosis in a 4-year-old girl:A case report  

作　　者：Jia-Wei Yao Li Qiu Ping Liang Han-Min Liu Li-Na Chen 

机构地区：[1]Division of Pediatric Pulmonology,Chengdu Women's and Children's Central Hospital,School of Medicine,University of Electronic Science and Technology of China,Chengdu 610091,Sichuan Province,China [2]Division of Pediatric Pulmonology and Immunology,Key Laboratory of Birth Defects and Related Diseases of Women and Children,Ministry of Education,West China Second University Hospital of Sichuan University,NHC Key Laboratory of Chronobiology,Chengdu 610041,Sichuan Province,China

出　　处：《World Journal of Clinical Cases》2022年第16期5380-5386,共7页世界临床病例杂志

基　　金：Supported by Science and Technology department of Sichuan Province,No.2020YFS0105;West China Second University Hospital of Sichuan University,No.KL036.

摘　　要：BACKGROUND Pulmonary lymphomatoid granulomatosis(PLG)is a lymphoproliferative disease associated with Epstein-Barr viral infection occurring mainly in adults and rarely in children.It is characterized by multiple pulmonary nodules.Its diagnosis depends on lung biopsy findings.Most patients are immunodeficient,and it commonly presents in children undergoing chemotherapy for leukemia.We report the case of a child with PLG caused by a mutation in the macrophageexpressed gene 1(MPEG1),suggesting possible PLG occurrence in children undergoing treatment for pulmonary nodular lesions.CASE SUMMARY This study reports a case of PLG without apparent immunodeficiency,suggesting the possibility of this disease occurrence during the treatment of pulmonary nodular lesions in children.Initially,the cause was assumed to be an atypical pathogen.Following conventional anti-infective treatment,chest computed tomography findings revealed that there were still multiple nodules in the lungs.Additionally,the patient was found to be infected with the Epstein-Barr virus.Histopathological examination of the resected lung revealed lymphoproliferative lesions with necrosis.Small lymphocytes,plasma cells,and histiocytes were observed in the background,although Reed-Sternberg cells were absent.Immunohistochemical staining[CD20(+),CD30(+),and CD3(+)]and EBV-encoded small RNA1/2 in situ hybridization of small lymphocytes revealed approximately 200 cells/high-power field.Whole exon sequencing of the patient revealed a mutation in the MPEG1.The patient was eventually diagnosed with PLG and transferred to the Department of Pediatric Oncology for bone marrow transplantation.CONCLUSION As PLG is rare and fatal,it should be suspected in clinical settings when treatment of initial diagnosis is ineffective.

关 键 词：Pulmonary lymphomatoid granulomatosis CHILD Epstein-Barr virus Lymphoproliferative disease LEUKEMIA Case report 

分 类 号：R734.2[医药卫生—肿瘤]