Treatment And Prognosis Of Peripheral T-cell Lymphoma

Part Ⅰ:Clinical and prognostic differences between ALK-negative anaplastic large cell lymphoma and peripheral T-cell lymphoma,not otherwise specified:A single institution experiencePurpose:Clinical differences between anaplastic lymphoma kinase-negative anaplastic large-cell lymphoma(ALK-ALCL)and peripheral T-cell lymphoma,not otherwise specified(PTCL-NOS)remain unclear.The aim of this study was to compare the clinical and prognostic features of the two entities.Patients and Methods:A total of 167 patients with ALK-ALCL(n = 48)and PTCL-NOS(n = 119)were reviewed.Patients with early-stage disease received chemotherapy with or without radiotherapy,whereas patients with advanced-stage disease received primary chemotherapy.Results:Compared with ALK-ALCL patients,PTCL-NOS patients exhibited distinct differences in clinical features with a propensity for more advanced stages,frequent extranodal involvement,and a poor performance status,leading to a higher risk group according to the International Prognostic Index or Prognostic Index for PTCL-NOS.Patients with ALK-ALCL were associated with a higher complete response rate(47.9%vs.31.0%;P = 0.041)after initial chemotherapy than patients with PTCL-NOS.The prognosis was significantly different between two subtypes,with a 5-year overall survival(OS)rate of 57.9%for ALK-ALCL and 23.9%for PTCL-NOS(P = 0.002).The subgroup analysis showed significant differences in OS and progression-free survival between the two subtypes in early-stage diseases,but not in advanced-stage diseases.Conclusion:Patients with ALK-ALCL showed favorable clinical features,higher chemosensitivity,and a superior outcome than those with PTCL-NOS.Part Ⅱ:Treatment outcomes of early stage peripheral T-cell lymphoma,not otherwise specifiedPurpose:The optimal therapy and prognosis of early stage peripheral T-cell lymphoma,not otherwise specified(PTCL-NOS)are not well defined.This study was conducted to evaluate the treatment outcomes in patients with early stage PTCL-NOS.Patients and Methods:Fifty patients who were diagnosed with early stage PTCL-NOS between January 1998 and December 2014 were reviewed.There were 19 and 31 patients with stage I and stage Ⅱ diseases,respectively.Of these patients,31 received radiotherapy with or without chemotherapy(RT w/o CT),18 received chemotherapy alone.Of patients receiving chemotherapy,21 were treated with CHOP(cyclophosphaminde,doxorubicin,vincristine,prednisone)and 16 with CHOEP(CHOP plus etoposide).Results:The 5-year overall survival(OS),cancer-specific survival(CSS)and progression-free survival(PFS)for all patients were 32.0%,34.9%and 27.6%,respectively.The overall response rate was 75.5%,with 61.2%complete remission(CR)rates.Radiotherapy significantly improved the prognosis compared with chemotherapy alone.The 5-year CSS rate was 44%for RT w/o CT,compared with 13%for chemotherapy alone(P = 0.045).The 5-year PFS rate was 41%and 13%(P = 0.044).Patients treated with CHOEP regimen were more likely to have adverse clinical features including ECOG score>2(P = 0.036)and elevated LDH(P = 0.001).However,there was no significant difference between CHOEP regimen and CHOP regimen in OS(3-year OS,41.4%versus 43.0%;P = 0.521)and PFS(3-year PFS,28.3%versus 29.7%;P = 0.986).Conclusion:The inclusion of radiotherapy has a significant impact on outcome for early-stage PTCL-NOS.Considering its aggressive clinical course and poor outcome,more effective systemic treatment strategies are urgently needed.Part Ⅲ:Radiotherapy is essential after complete response to initial chemotherapy in early-stage extranodal nasal-type NK/T-cell lymphoma:analysis from a multicenter studyPurpose:This study aimed to clarify the treatment outcomes of chemotherapy(CT)alone and assess the value of radiotherapy(RT)in patients with early-stage extranodal nasal-type NK/T-cell lymphoma who achieved a complete response(CR)after initial CT.Patients and Methods:A total of 217 patients achieved CR after initial CT;160 received additional RT(CT+RT),57 did not receive RT(CT alone).A comparative study was performed using propensity score matching(PSM).The cumulative incidence of locoregional recurrence(LRF)and systemic failure(SF)were investigated with competing risks method.Results:CT alone resulted in 5-year progression-free survival(PFS)and overall survival(OS)rates of only 37.9%and 58.8%,respectively;CT+RT significantly improved PFS(70.0%,P<0.001)and OS(79.1%,P = 0.011).After PSM,CT+RT still resulted in significantly better survival outcomes than CT alone.The beneficial survival effects of RT were observed in all subgroups,including patients receiving at least four CT cycles or L-asparaginase-based regimens.Furthermore,CT alone resulted in frequent disease recurrence;additional RT provided a survival benefit by reducing both LRF(5-year cumulative LRF,45.1%vs.14.4%,P<0.001)and SF(5-year cumulative SF,33.0%vs.20.1%,P = 0.046).In multivariate analysis,additional RT remained an independent prognostic factor for recurrence and survival.Conclusion:For patients with early-stage extranodal nasal-type NK/T-cell lymphoma who achieve a CR after CT,CT alone results in high rates of recurrence and poor prognosis;RT is essential to improve disease control and survival.These findings provide important evidence for treatment decision-making and design of prospective clinical trials.