| Objective:The aim of this study was to analyze the clinical features and prognostic of follicular lymphoma(FL)with diffuse large B cell lymphoma(DLBCL)components(FL/DLBCL)or transformed follicular lymphoma(t-FL),as well as those with pure FL or de novo DLBCL as controls.Methods:46 cases of FL/DLBCL patients and 17 cases of t-FL patients were collected in the union hospital affiliated to fujian medical university from January 1,2012 to June 1,2019,as well as those with pure FL(n=131)or de novo DLBCL(n=365)at the same period as controls,all patients were confirmed by histopathological examination.Demographic data,clinical characteristics,major laboratory results,treatment regimen,treatment outcome and prognosis of all patients were recorded.The differences between different groups were compared and the prognosis factors was analyzed by univariate and multivariate analysis.Results:1.In the 46 cases of FL/DLBCL,33 males and 13 females,with a median age of53.5(rang 22 to 87)years.The patients showed the following grading of the FL component:grade 1 to 2,1 case(2.2%);grade 3a,20cases(43.5%);grade 3b,17 cases(37.0%)and grade 3,8 cases(17.4%).Among FL/DLBCL cases,29(65.9%)were of GCB origin,15(34.1%)non-GCB and 2 unclassified.The median proportion of the DLBCL component was 50%(rang 15%to 99%).Ki-67 value of FL/DLBCL patients is between FL 1-3a and DLBCL(P<0.001).Compared with FL1-3a cases,patients with FL/DLBCL showed more frequently B symptoms and high LDH level(P<0.05).Compared with DLBCL cases,patients with FL/DLBCL showed more frequently GCB(P=0.004).2.In the 17 cases of t-FL,11 males and 6 females,with a median age of 56(rang29 to 89)years.Among t-FL cases,10(66.7%)were of GCB origin,5(33.3%)non-GCB and 2(4.3%)unclassified.5 cases(29.4%)of histologic transfomation occurred within the second year of follow-up.The median histological transformation time of all patients was 59(rang 5 to 246)months.Genetic test data were obtained in 10 patients(58.8%),including 2 cases with BCL-2 gene positive,1 case with IGH/BCL-2 gene positive,1 case with C-MYC and BCL-2 gene positive,1 case with C-MYC,BCL-2 and BCL-6 gene positive,1 case with TCR-βgene positive,1 case with TP53 gene positive,5cases(50%)with BCL-2 gene abnormality.Before and after histologic transformation,3and 6 patients were able to obtain chromosomal data,and no abnormalities were found.Compared with FL1-3a cases,patients with t-FL showed more frequently B symptoms,hepatosplenomegaly,high LDH level,anemia,highβ2-Microglobulin level,hypoalbuminemia and extranodal involvement>1(P<0.05).3.All cases of FL/DLBCL were treated like DLBCL,none received further intensive treatment,the complete response rate and overall response rate were 60%(24/40)and 87.5%(35/40),respectively.With a median follow-up time of 36 months,22.9%(8/35)patients relapsed.The overall response rate and recurrent rate of FL/DLBCL patients were not statistically significant with FL1-3a and DLBCL patients.The PFS and OS of FL/DLBCL patients were similar to DLBCL(PFS:χ~2=0.370,P=0.543;OS:χ~2=1.067,P=0.302)and worse than FL1-3a(PFS:χ~2=5.206,P=0.023;OS:χ~2=10.081,P=0.001).The grade of the FL component,the cell origin of the DLBCL component,the proportion of DLBCL component did not impact on PFS and OS of FL/DLBCL patients(P>0.05).4.All cases of t-FL were treated with immunochemotherapy,two patients received CAR-T therapy and one received ASCT.The complete response rate and overall response rate were 13.3%(2/15)and 53.3%(8/15),respectively,which were worse than FL/DLBCL and DLBCL(P<0.05).With a median follow-up time of 10 months,50%(4/8)patients relapsed.The median PFS was 15 months(95%CI:5.577-24.423),which were worse than FL/DLBCL(χ~2=7.408,P=0.006)and DLBCL(χ~2=6.901,P=0.009).The median OS was 27 months(95%CI:5.114-48.886),which were worse than FL/DLBCL(χ~2=7.302,P=0.007)and DLBCL(χ~2=6.389,P=0.011).5.Univariate analysis showed that HB,clinical stages and FLIPI score were significantly associated with PFS in FL/DLBCL;but HB,ALB and FLIPI score were significantly associated with OS in FL/DLBCL.No independent risk factors affecting the prognosis of FL/DLBCL patients were found in multivariate analysis.Conclusion:1.The majority of DLBCL components in FL/DLBCL patients were GCB-like subtypes.The PFS and OS of FL/DLBCL patients is worse than that of pure FL1-3a,but is not worse than that of de novo DLBCL.FL/DLBCL patients should be treated with immunochemotherapy as aggressive lymphoma,however,such intensive therapies as hematopoietic stem cell transplantation need to be further explored.2.Half of the t-FL patients in this study had BCL-2 gene abnormality.Compared with pure FL1-3a,patients with t-FL showed more frequently B symptoms,hepatosplenomegaly,high LDH level,anemia,highβ2-Microglobulin level,hypoalbuminemia and extranodal involvement>1.The overall response rate,PFS and OS of t-FL patients are worse than FL/DLBCL and DLBCL patients. |
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