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Clinical Characteristics Of Lymphoma With Mediastinal Masses

Posted on:2019-07-13Degree:DoctorType:Dissertation
Country:ChinaCandidate:H YuFull Text:PDF
GTID:1364330545484069Subject:Internal Medicine
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Part One Analysis of clinical characteristics of 26 patients with primary mediastinal large B cell lymphomaObjectiveThis study was aimed to elucidate the clinical manifestation,treatment regimens and related prognosis factors of primary mediastinal large B cell lymphoma(PMBCL).MethodThe clinical data of 26 PMBCL patients admitted in the first affiliated hospital with Nanjing medical university(Jiangsu province hospital)from February 2007 to January 2018 were retrospectively summarized,the relevant data were analyzed and literature review was conducted.ResultsOf the 26 patients,10 were male,16 were female,and the ratio was 1:1.6.The median age was 28(18?46)years old.The main clinical features included mediastinal bulk mass(44.0%),superior vena caval syndrome(69.2%),cough(46.2%),serous membrane fluid(40%),thoracalgia(11.5%),edema of face and neck(15.4%),and dysphagia(11.5%).According to Ann Arbor staging system,12 cases(46.2%)were classified to stge ?/? and 14 cases(53.8%)to stage ?/?,7 patients(26.9%)had B symptoms.All patients were treated with rituximab(R)conbined with chemotherapy(immuno-chemotherapy),8 patients conbined with involved field radiotherapy(IFRT),7 patients conbined with autologus stem cell transplatation(ASCT),2 patients received both IFRT and ASCT.The median immuno-chemotherapies were 7(1?16).Five patients received R-CHOP as frist-line regimen,4 of them received IFRT,1 patient received both IFRT and ASCT.Nineteen patients received R conbined with dose ajusted EPOCH(R-DA-EPOCH)as frist-line regimen,4 of them received IFRT,6 of them received ASCT,1 patient received both IFRT and ASCT.The median follow-up were 51.5(2?133)months.Of the 25 patients whose efficacy could be evaluated,21(84.0%)cases achieved complete remission(CR),8 cases(8.0%)achieved partial remisson(PR),1 patients(8.0%)achieved stable disease(SD),and 1 patient experienced progressive disease(PD)and died eventually.The 5-year progression free survival(PFS)and overall survival(OS)were 83%and 95.7%,respectively.The rate of radiotherapy in R-DA-EPOCH cohort was lower compared with R-CHOP cohort(80%vs.21.1%,p=0.028),and CR rate was higher(40%vs.94.4%,p=0.021).Univariate anylasis showed that pericardial effusion was a poor prognostic factor for OS,and first-line therapy of R-DA-EPOCH had PFS(p=0.001)and OS(p=0.039)advantage over R-CHOP regimen.ConclusionPMBCL usually affects young adults with a female propensity,and has unique clinical features with good prognosis.Pericardial effusion was a poor prognostic factor for OS.R-DA-EPOCH immunochemotherapy is an effective regimen.The optimal treatment for PMBCL remains to be further explored in prospective clinical trials.Part Two Lymphoma with mediastinal masses:Analysis of clinical characteristics of 30 patientsObjectiveTo analyze the incidence,clinical characteristics,immunohistochemistry(IHC)features,treatment modalities and progonosis of lymphoma patients with mediastinal masses,except primary mediastinal large B cell lymphoma(PMBCL).MethodsThe clinical data of 30 lymphoma patients with confirmed pathological diagnosis who were admitted in the first affiliated hospital with Nanjing medical university(Jiangsu province hospital)from February 2007 to January 2018 were retrospectively analyzed.The general conditions,clinical manifestations,involved sites,IHC,laboratory examinations,treatment regimens and efficacy,and prognostic factors were analyzed.ResultsAmong the 30 patients,male and female were are equally represented,accounting for 15(50%)cases,respectively.The median age was 31.5(17?72)years old,25(83.3%)patients aged below 40 years,and 29(96.7%)patients aged below 60 years.All 30 patients were pathologically diagnosed,among which,9(30.0%)cases were diffuse large B cell lymphoma,not otherwise specified(DLBCL,NOS),17(56.7%)cases were classical Hodgkin's lymphoma(cHL),and 4 cases(13.3%)were primary mediastinal grey zone lymphoma(MGZL).With respect to IHC,CD20 expression rate were 3/16(18.8%),2/3(66.7%),9/9(100%)in cHL,MGZL and DLBCL,NOS cohorts,respectively.CD15 expression rate were 13/17(76.5%),1/3(33.3%),0/3(0),respectively,and CD30 expression rate were 16/17(94.1%),3/3(100%),2/5(40%),respectively,in the aformentioned three cohorts.EBER was positive in 1/12(8.3%)cHL.All patients were treated with chemotherapy.In cHL cohort,10 cases conbined with involved field radiotherapy(IFRT),6 patients conbined with autologus stem cell transplatation(ASCT),4 patients received both IFRT and ASCT,10(58.8%)cases achieved complete remission(CR),2(11.8%)cases achieved partial remisson(PR),and 5(29.4%)patients experienced progressive disease(PD),the 1-,2-,and 5-year overall survival(OS)rates were 94.1%,88.2%,and 67.2%,respectively.In DLBCL,NOS cohort,3 cases conbined with IFRT,2 cases with ASCT,1 patient received both IFRT and ASCT,4(44.5%)cases achieved CR,2(22.2%)cases achieved PR,1(11.1%)case was stable disease(SD)and 2(22.2%)cases experienced PD,the 2-,5-year OS rates were both 77.8%.In MGZL cohort,3 cases conbined with IFRT,1 case with ASCT,1 case received both IFRT and ASCT,2(50%)cases achieved CR,2(50%)cases experienced PD,the 1-,2-,5-year OS rates were 66.7%,66.7%,and 33.3%,respectively.Including PMBCL,there was a significant difference of OS among these 4 entities(p=0.026).In intra-group comparison,OS of PMBCL group were significantly longer than that of MGZL group(p=0.001).ConclusionMediastinal masses are characterized by unique clinical features due to their unique anatomical location.Several lymphomas which manifested primary or secondary mediastina involvement,including cHL,MGZL,and DLBCL,NOS tends to occur in young adults,and have overlapping and transitional changes in immunophenotype.The prognosis of PMBCL is prior to MGZL.
Keywords/Search Tags:Primary mediastinal large B cell lymphoma, clinical characteristics, prognosis, classical Hodgkin's lymphoma, primary mediastinal grey zone lymphoma, diffuse large B cell lymphoma, not otherwise specified, immunophenotype
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