Analysis Of Clinicopathologic Features Of Lymphomatoid Granulomatosis

Background and ObjectiveLymphomatoid Granulomatosis(LyG)is a kind of Epstein-Barr virus(EBV)related Lymphoproliferative disease,which mainly invades the tissues and organs outside the lymph node.The disease was first discovered in 1972 when Wegener’s granuloma was studied by Liebow et al,and it was described as a kind of disease with pleomorphism,vascular centrality and vascular invasiveness with necrotizing characteristics,as well as atypical lymphatic reticular endothelial cell infiltration..More than 80% of the disease involve in the lung,but also involve in the other tissues and organs such as the central nervous system,skin,kidney and so on.The disease is highly heterogeneous,and as a rare lymphocyte proliferative disease associated with EBV,LyG can develop from benign lymphocytic proliferative disease to malignant lymphoma during the period of the disease,showing atypical imaging characteristics and different clinical symptoms according to the different sites of invasion.Pathological examination is an important clue for the diagnosis of this disease.However,because there are often many inflammatory cells in the background and rare typical tumor cells with varying degrees of necrosis,and clinical manifestations or pathological features are not specific,which may easily lead to missed diagnosis and misdiagnosis.In addition,the overall incidence of the disease is low,so most domestic and foreign literatures are reported in the form of case report and the understanding of the disease is not comprehensive.This study summarized some cases from our center and the literature both at home and abroad,analysed the patient’s general condition,past medical history,clinical manifestation,pathological features,the choice of current treatment and living conditions,in order to improve theclinical doctors to LyG understanding,reduce missed diagnosis rate and misdiagnosis rate,which can provide more basis for the further research of the disease and the way of thinking.MethodThe clinical features,pathological findings and treatment of 6 patients with lymphomatoid granulomatosis admitted to the First Affiliated Hospital of Zhengzhou university were retrospectively analyzed and summarized.Meanwhile I manually retrieved the common database such as Wanfang Medine,China National Knowledge Infrastructure(CNKI)as well as Pubmed database commonly used abroad about cases of LyG.Finally,according to the inclusion and exclusion criteria formulated in this paper,literature reports at home and abroad including 84 cases were sorted out and analyzed.Besides,the general situation,clinical manifestations,pathological characteristics,diagnosis,treatment regimens,survival and other data of this disease were counted.In addition,Statistical processing software of SPSS20.0 was used to summarize and analyze the collected data.ResultsBy reviewing and analyzing the cases in our hospital and the literature reports in the past 10 years,a total of 84 patients were included according to the established inclusion and exclusion criteria,and the characteristics of the disease were summarized as follows :(1)The incidence of LyG in males was higher than that in females,and mostly involved in the middle-aged and elderly people,sometimes LyG could also been in children.The median age of onset is 54 years old,and the status of immune function had a certain relationship with the disease;(2)Lesions of LyG could involve the whole body,most of which were found in the lungs,especially the bilateral lower lungs,and they were also could be found in the central nervous system,skin,liver,adrenal gland,etc.,with the non-specific clinical manifestations.Skin manifestations were more common in foreign cases.(3)Typical pathological manifestations of LyG were mainly the invasion and destruction of blood vessels.Under the background of lymphocyte composition,different numbers of atypical B cells could be found,which can be divided into 3grades according to their pathological grades,namely,proliferative,borderline and neoplastic lesions.(4)The treatment regimen of LyG was not uniform.And the treatment protocol was mainly individualized based on the patient’s immune status,lesion site,clinical manifestations and pathological grading.The common treatment regimen was chemotherapy,and R±CHOP was the main option.(5)The median survival time of the patients with LyG in this group was 12 months.In the univariate prognostic analysis,it was found that the lesions occurred only in the central region(P=0.044)and the EBER negative(P=0.030)had relatively good overall survival,but no significant independent prognostic indicators were found in the multivariate analysis.Conclusion1.LyG tends to involve middle-aged and elderly people and most commonly invade the lung.Immune dysfunction is an important risk factor for the disease.2.LyG is a kind of rare disease with nonspecific clinical manifestations,pathological features and imaging manifestations.It is prone to missed diagnosis and misdiagnosis.Attention should be paid to differential diagnosis with the common diseases.3.LyG treatment regimen is relatively inconsistent,and R±CHOP regimen is the main chemotherapy regimen.4.The overall prognosis of LyG is poor,and prognostic factor need to be studied further.