| Objective:Peripheral T cell lymphoma(PTCL)is a clinically rare,heterogeneous and complicated malignant tumor of the blood system with a poor prognosis.In 2017,WHO has identified about 30 PTCL subtypes,and in the process of tumor differentiation of PTCL,the B lymphocytes and myeloid transformation,and even the secondary tumors can occur.The biological characteristics of each PTCL subtype are different.In this paper,the clinical characteristics,therapeutic efficacy and survival of 39 patients with PTCL admitted to our hospital were retrospectively analyzed to provide theoretical basis for the diagnosis and treatment of this disease.Methods: According to the classification standard of PTCL subtype updated by WHO in2017,A retrospective analysis was performed on 39 PTCL patients with complete clinical data who were admitted to the first affiliated hospital of China medical university from January 2013 to June 2019.The clinical information of the patients was sorted out and stratified in detail,the general clinical characteristics and bone marrow involvement were elaborated,the post-treatment remission rate,survival status and the risk factors affecting prognosis were evaluated,and the clinical characteristics were summarized and analyzed based on the relevant literature reports.Results:1.General clinical characteristics: Among the 39 patients with PTCL,12 patients with PTCL-NOS,12 patients with ENKTL,7 patients with AITL,6 patients with ALCL,and 2 patients with SPTCL.The male to female ratio was 1.44:1,and the median age of onset was 53(14-77)years.The first symptoms: 23 cases(59%)of intrarenal lymphoma had enlarged lymph nodes.The 12 cases of ENKTL were mainly local symptoms of nasal cavity.2 cases of cutaneous ALCL and 2 cases of SPTCL showed skin features such as rashes on trunk and limbs,nodules around eyes and lips,and penile masses.When the 39 patients were initially diagnosed,18(46.2%)had symptom B,16(41.0%)had splenomegaly,13(33.3%)had stage iv,6(15.4%)had prognostic index ?3points,7(17.9%)had tumor maximum diameter ? 5cm.LDH was increased(>225u/l)27 cases(69.2%),?2-MG was increased(>1.8 mg/l)37 cases(94.9%).2.Bone marrow involvement:There were 8 cases of bone marrow involvement and 6cases of T cell bone marrow infiltration(2 cases each of PTCL-NOS,AITL andENKTL).Two cases of PTCL were accompanied by abnormal marrow/b-line cell infiltration(PTCL-NOS combined with MPAL,ENKTL combined with B-NHL).Survival analysis showed that the survival time of patients with abnormal myeloid /B cells with bone marrow infiltration was shorter than that of patients with T cells infiltration(P=0.004),with an average OS of 4.3(95% ci0-8.7)months.3.Remission after treatment of all subtypes : The total disease control rate(DCR)(CR+PR+SD),total therapeutic response(CR+PR)and complete response rate of the 39 patients were 74.4%,61.5% and 38.5% respectively.The ORR of ALCL was higher which was 100%(P=0.041),while the other subtypes were 50-60% with no statistical difference.In particular,12 patients with ENKTL subtype without stage IE,l-asp or peg-asp combined chemotherapy and radiotherapy were used as appropriate,and the ORR was 50%.Among them,5 patients with extra-nasal multisite stage IV involvement progressed rapidly(including 2 patients with concurrent HPS1 month death),that is,the PD rate was higher at 41.7%.4.Efficacy of different chemotherapy regiments: CHOP ORR was 71.4% in 14 patients.The ORR of 9 patients receiving vp-16 combined with chemotherapy was66.7%.2 cases of PTCL-NOS(extensive metastasis,tumor diameter ?5cm,splenomegaly,bone marrow involvement)receiving the regimen of Hyper-CVAD chemotherapy progressed rapidly.There was no significant difference in ORR between different treatment regimen.The survival time of the Hyper-CVAD chemotherapy group was significantly lower than that of the other two groups(P=0.000).Chidamide is expensive,and only 2 patients(1 case each of PTCL-NOS and SPTCL)received this drug in combination with chemotherapy after ineffective chemotherapy alone,and all obtained PR.All 3 patients(1 case each of SPTCL,ALCL,ENKTL)received ASCT with significant efficacy,and the longest progression-free survival(PFS)was up to 29 months.One patient with ALCL with recurrent bone marrow involvement received ASCT and did not reach CR.CR was obtained by allo-hsct in sequence,and the patient was still alive after 72.5 months of follow-up.5.Survival analysis: The median follow-up of 39 patients was 22(1-72.5)months,and the median OS did not reach.The mean OS was 49.1(95% ci38.1-60.2)months,and the estimated 3-year survival rate was 69.1%(95% ci53.6-84.6%).The prognosis of 12 ENKTL patients was poor,with an average OS of 19.3(95% ci10.5-28.1)months,a median OS of only 9.5 months,and a 3-year survival rate of 47.6%.The remaining subtypes survived for a relatively long time.6.Prognostic analysis: PS score ?2 points and recent chemotherapy failure were independent risk factors affecting the long-term survival of patients in this group,with the risk of death 7.014 times and 49.944 times,respectively.Conclusion: PTCL is a blood system tumor with significant heterogeneity.The most common clinical typing was ptclnos and ENKTL(30.8%),with a high incidence rate in males(1.44:1 for males to females)and a late onset age(median onset age was 53years).Its biological behavior is highly invasive,and it has a high proportion of extraterritorial tumors(41.0%),suggesting that clinicians should be alert to different tumor sites and make correct diagnosis at the early stage of the disease.Bone marrow involvement(20.5%)was common,and even PTCL with abnormal myeloid/b-line bone marrow infiltration was found,whose survival time was significantly lower than that of patients with t-cell bone marrow infiltration(P=0.004).The simultaneous development of multiple hematologic tumors was associated with poor prognosis,and it was urgent to develop a treatment method for multiple hematologic malignancies.In terms of treatment,the efficacy of traditional chemotherapy was not ideal,and the total remission rate of PTCL(61.5%)was still low.The new drug Chidamide may yield better efficacy.Combined with hematopoietic stem cell transplantation,the overall survival time may be further extended.The clinical process and treatment principles of ENKTL were significantly different from those of other subtypes.External nasal involvement in multiple sites and complicated HPS progressed rapidly with a high fatality rate.Generally,the prognosis of patients with unlocal-limited ENKTL was poor.In terms of prognosis,PS score ?2 points and the inefficacy of recent chemotherapy was an independent risk factor for the prognosis of patients.More well-designed clinical studies are needed to explore new strategies to overcome drug resistance in order to improve patient survival. |
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