Analysis Of Clinical Characteristics Of Enteropathy-Associated T-cell Lymphoma

Background and ObjectiveEnteropathy-associated T-cell lymphoma(EATL)is a rare tumor that originates from intestinal T cells and is highly invasive.A lot of evidence shows that it is closely related to the complications of celiac disease.The small intestine is most often invaded,followed by the colon.The main clinical manifestations of EATL were malabsorption,abdominal pain,diarrhea,acute abdomen and fever.With the emergence of new data on etiology and epidemiology,the World Health Organization(WHO)classified EATL into type Ⅰ and type Ⅱ.Type Ⅰ EATL is closely related to the incidence of celiac disease,and its high incidence is in northern Europe with a high incidence of celiac disease.while type II EATL is not associated with celiac disease,and its incidence is relatively high in Asia.The classification of these two types is controversial,so type II EATL was separately named as monomorphic epitheliotropic intestinal T-cell lymphoma(MEITL)according to WHO classification in 2016.Later studies have also proved that,in addition to epidemiological and pathogenic characteristics,the differences in pathological and molecular pathological features tend to be an independent subtype of MEITL.The clinical manifestations of MEITIL are not typical and varied,so it is easy to lead to clinical misdiagnosis and delay early diagnosis and treatment.In addition,as a rare disease with very low incidence,most of the literature reports at home and abroad are case reports.From the results reported in the literature,the effect of traditional chemotherapy on MEITL is not good,and the prognosis is very poor.Hematopoietic stem cell transplantation may be an option to prolong the survival time of patients,but due to the rapid progress of MEITL and serious complications,most patients do not meet the criteria.This paper summarizes the cases reported in the literature of type II EATL/MEITL in our hospital and at home and abroad,in order to improve the clinicians’ understanding of it and provide more basis and ideas for its further research.Materials and methodsWe summarized the clinical data of 17 patients with type II EATL/MEITL treated in the first affiliated Hospital of Zhengzhou University from 2010 to 2020 and searched 52 cases of type II EATL with complete clinical data and survival information reported at home and abroad.The general data,on-the-spot manifestations,pathological features,treatment plans and survival data of these cases were analyzed retrospectively,and the SPSS21.0 software was used for statistical analysis and summary.ResultThrough the summary and analysis of 69 cases in our hospital and cases reported in the literature,the characteristics of the disease are as follows:1.Seventeen cases of enteropathy-associated T-cell lymphoma treated in our hospital were type II,and 69 cases of type II EATL/MEITL had no history of celiac disease.2.No matter the cases of type II EATL in our hospital or the cases reported in the literature,the incidence of male was more than that of female.The median age of onset was 53 years old,with an average of(51.7±14.9)years.3.The main clinical manifestations were weight loss,diarrhea,abdominal pain,fever,abdominal distension and gastrointestinal bleeding;some patients were accompanied by intestinal obstruction and intestinal perforation,and more than half of the patients had B symptoms.4.The main sites of invasion are jejunum,ileum,colon,and can also be seen in other parts of digestive tract,such as rectum,stomach,esophagus and so on.Lung invasion of organs outside the digestive tract is the most common,and other organs are rare.5.Tumor cells are usually positive for CD3,CD8,CD43,CD56,TIA-1 and GranzymeB,but negative for CD4,CD5 and CD30,negative for EBER in situ hybridization,and most of them are more than 50%Ki-67.6.The median survival time of 69 patients was 9(0.33-60)months.The main alternative treatments are chemotherapy ±surgery and hematopoietic stem cell transplantation after chemotherapy.Compared with simple operation,chemotherapy alone(13.380),chemotherapy combined with surgery(22.592),and transplantation after chemotherapy(15.488)can make patients survive longer than those who received surgery alone(chemotherapy 13.380,0.000),combined with chemotherapy(22.592,22.592,0.000),and transplantation after chemotherapy(15.488,15.488,0.000)can make patients have a longer survival time.Whether surgery had no effect on OS(0.096),chemotherapy could significantly prolong the survival time of patients compared with non-chemotherapy(chemotherapy 15.074～0.000),and the survival time of patients with chemotherapy was significantly longer than that of patients without chemotherapy(P<0.05),and the survival time of patients with chemotherapy was significantly longer than that without chemotherapy.Conclusion1.Type Ⅱ EATL/MEITL is mainly in middle-aged and elderly men,and the most common site is the small intestine.2.The prognosis of MEITL/type Ⅱ EATL is very poor,the median OS is less than 1 year,and the prognostic factors need to be further studied.3.Gemcitabine/platinum-based chemotherapy combined with ASCT is the firstline treatment choice for MEITL/type Ⅱ EATL,surgery cannot prolong the OS.