| Objective:Primary mediastinal large B cell lymphoma(PMBCL)is a special type of B cell lymphoma originating from the medulla of the thymus.Due to its clinical,pathological and genetic characteristics,it is considered as an independent type.According to the classification of WHO in 2008,primary mediastinal large B cell lymphoma has been listed as a substype of the mature diffuse large B cell lymphoma.Mediastinal large B cell lymphoma accounts for2-4%of the non Hodgkin lymphoma.6-10%of diffuse large B cell lymphoma.PMBCL is especially prevalent in young women aged 30-40 year-old.The diagnosis of primary mediastinal large B cell lymphoma is difficult because of its low incidence and atypical symptoms,this study aimed at investigating the clinical characteristics,pathologic features,treatment and prognostic factors of PMBCL to provide clinical data for the establishment of standardized treatment scheme.Methods:Our study collected clinical data of 36 patients from our hospital diagnosed as PMBCL by mediastinum biopsy and immunohistochemistry,from January 2010 to January2017,analyzed their clinical features,pathological characteristics,and treatment?prognostic factors.Ann Arbor method was used in clinical staging,patients'general condition was evaluated by Eastern Cooperative Oncology Group(ECOG)evaluation standards.We made prognosis grouping with reference to the International Prognostic Index(IPI)score,the data was analyzed by SPSS19.0 software.The survival rate and factors affecting the prognosis was calculated by Kaplan Meier method.Treatments were compared using?~2test,with P<0.05 as the standard when the difference was statistically significant.Result:The median age of these 36 patients with PMBCL is 39-years old,main manifestations are superior vena cava syndrome(SVC)(52.8%),http://fanyi.baidu.com/-zh/en/javascript:void(0);Followed by pleural effusion in 13 cases(36.1%),cough in 11 cases(30.6%),dyspnea in 5 cases(13.9%),dysphagia in 5 cases(13.9%),and 2 cases involving bone marrow(5.6%).According to the Ann Arbor stage criterion,there were 24 cases(66.7%)in stage I-II and 12 cases(33.3%)in stage III-IV.6 cases(16.7%)were combined with B symptom.After treatment,13 cases reached complete remission,15 cases reached partial remission,5 cases were stable disease,2 cases were progressive disease.1 case who received surgery alone doesn't have data of evaluation?Till the end of follow-up,The median survival time is 37 months,the overall survival rates of 2-years,3-years,4-years were 83.3%,76.2%,68.8%and 56.25%respectively.The 2-year survival rate of R-CHOP group?CHOP group?R-DA-EPOCH group and CHOPE group were 91.7%?100%?85.7%,and 66.7%respectively.4 cases who received stem cell transplantation are alive till the end of follow-up.the loss of follow-up is 3,and 5 cases died(among which 1 case relapsed,other 4cases were due to progression of disease).The patients'age,sex,superior vena cava syndrome,pleural effusion,the size of the mediastinal mass,LDH level,B symptom,Ann Arbor staging,the ECOG score and so on were used for the analysis by Kaplan Meier test.The results showed that the level of LDH(higher than the upper limit 2 times)and the size of mediastinal mass were the prognostic factors(P<0.05).Conclusions:The PMBCL has younger median age,and the stages are usually early due to mediastinal compression syndrome.Prominent symptoms include superior vena cava obstruction syndrome,cough,dyspnea,dysphagia,and rare bone marrow involvement.The size of mass and LDH level are prognostic factors,rituximab combined with chemotherapy has definite therapeutic effect,mediastinal radiotherapy should be used as supplementary treatment,Stem cell transplantation is of great significance for the treatment of relapse and refractory disease. |
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