| Background and purposeExtranodal natural killer/T-cell lymphoma(ENKTCL)is a highly aggressive Non-hodgkin's lymphoma(NHL).Incidence of Asia and Latin America is far higher than that of western countries.It accounts for 5% to 18% of NHL,first in T/NK cell lymphoma in China.The disease appears progressive development process.The survival period is short with a poor prognosis.According to the different anatomical site of primary lesions,ENKTCL is divided into the two subtypes of Upperaerodigestive tract ENKTCL(UAT-ENKTCL)and Non-upperaerodigestive tract ENKTCL(NUAT-ENKTCL).UAT-ENKTCL is highly clinical common,accounting for more than 80% of the ENKTCL.It mainly locates on the face of the midline,such as the nasal cavity and adjacent tissues of nasal,nasopharyngeal,oral and oropharyngeal etc.There is a lot of researchs on the type of clinical diagnosis and treatment.However,the incidence of NUAT-ENKTCL is very low,only accounts for 10% ~ 20% of ENKTCL,often infringe upon the intestinal tract,skin,testicular,lungs,liver,and other part of outside the nasal cavity.The proportion of patients with late malignant degree is higher,less response to therapy.Patients often die of disease progression and corresponding complication in a short term.Therefore,relevant clinical case studies are less reported.At present,few studies are reported about prognosis on NUAT-ENKTCL,mainly UAT-ENKTCL.Eastern Cooperative Oncology Group(ECOG)score,lactate dehydrogenase Dehydrogenase(LDH),albumin,disease stage,number of extranodal involvement,Hemoglobin(HB),EBV level can affect the prognosis.However,different literature reports are inconsistent.The principle of treatment is mainly based on UAT-ENKTCL or a small sample clinical analysis of specific site ENKTCL(such as the intestine,testis and lungs).Due to the lack of large sample clinical cases to observe,we lack sufficient knowledge of the clinical features,survival,prognosis,and treatment of this type of lymphoma.The clinical and pathological data of 66 patients with NUAT-ENKTCL treated in the First Affiliated Hospital of Zhengzhou University from May,2011 to July,2016 were collected.It mainly includeds sex,age,primary site,staging,ECOG score,IPI score,pretreatment,beta2-MG,LDH,WBC,hemoglobin,platelet,albumin,EBV levels.The purpose of this study was to summarize the epidemiology,clinical and pathological features of NUAT-ENKTCL,and to explore the prognostic factors and treatment strategies.Data and Methods1.Retrospective analysis of the pathologically confirmed 66 cases of non-upper respiratory tract digestive tract extra-nodal NK/T cell lymphoma clinical and pathological data in Zhengzhou University First Affiliated Hospital from May 2011 to July 2016.Of the 66 patients with NUAT-ENKTCL,there were 43 males and 23 females,accounting for 13.25% of the total ENKTCL in the same period.Among them,20 cases were primary the intestinal tract,16 cases of systemic multiple site violations,14 cases of primary skin and soft tissue,5 cases of lung,3 cases of esophagus,liver-spleen,testicular respectively,2 cases of adrenal gland.According to the Ann Arbor staging system,there were 12 cases of stage I,7 cases of stage II,3 cases of stage III,44 cases of stage IV and stage III+IV account for 86.4%.There were 48 patients with B symptoms,40 patients with extra-nodal involvement more than 2 sites,34 cases with ECOG score more than 2 points,39 cases with IPI score of 3-5 points.?2-MG increased in 40 cases.34 cases of patients combined with serous effusion and 32 cases with spleen-omegaly.Of the 28 patients tested,22 have a significant increase in EBV levels before treatment.CD56 was negative in 18 cases and Ki-67 more than 50% in 52 cases.30 patients were combined by severe complications,including 11 cases with hemo-phagocytic syndrome(HPS),gastrointestinal bleeding in 11 cases,10 cases of digestive tract perforation,septic shock in 9 cases,5 cases of multiple organ dysfunction syndrame,acute pancreatitis in 1 case.25 cases received chemotherapy among the 66 patients,11 cases of surgery alone,surgery combined with chemotherapy in 13 cases,symptomatic support in 15 cases.2.SPSS 21.0 was used to statistically analyze the clinical data and survival of the patients.Qualitative data were expressed by rate(%)and chi square was used between groups.Quantitative data were expressed by mean and standard deviation and t test was used between groups.Survival analysis used Kaplan-Meier survival analysis.The survival rate of the two groups of patients used Log-rank test;multi-factor survival analysis with COX proportional risk regression model and P= 0.05 was used as the test standard Results1.Clinicopathological features: There are 34 cases(51.52%)of primary intestinal tract and skin,30 cases with severe complications(45.46%).2.Survival analysis: At the follow-up date in December 2016(follow-up time of 10 days to 62 months),of the 66 cases of NUAT-ENKTCL patients,50 patients die,3 cases lost,13 cases survive.Kaplan-Meier survival analysis show that the median survival time of 66 patients with NUAT-ENKTCL is 4.0 months,the mean survival time is 17.688 months,the 1 year OS is 33.9%,the 2 year OS is 26.5%,the 3 year OS is 21.9%,5 year OS is 10.9%.The single factor survival analysis show that the IPI score(P=0.000),ECOG score(P=0.000),Ann Arbor staging(P=0.002),Primary site(P=0.033),the number of extra-nodal involvement(P=0.002),albumin(P=0.026),LDH(P=0.003),serous effusion(P=0.032),severe complication(P =0.000)and EBV(P=0.039)of 28 cases affect the survival prognosis of patients with NUAT-ENKTCL(P <0.05).However,the sex(P=0.553),B symptom(P=0.085),?2-MG(P=0.073),CD56(P=0.745),Ki-67(P=0.936),hemoglobin(P=0.684)and splenomegaly(P=0.869)have no statistically significant effect on survival rate(P<0.05).Multivariate analysis of the prognostic factors associated with prognosis is performed.The RR of LDH level is 2.363(P=0.017 <0.05);the RR of severe complication is 7.298(P=0.000 <0.05).which is significantly correlated the prognosis of NUAT-ENKTCL(P<0.05).3.The 1 year OS,2 year OS,3 year OS of chemotherapy group are 58.64%,54.45% and 44.68%,respectively and the combined surgery and chemotherapy are 50.48%,37.86%,12.62%,respectively.There are no significant difference between the two groups(P=0.116).Conclusion1.NUAT-ENKTCL is most common in intestinal and skin soft tissues.It often incorporates a variety of serious complications and have poor prognosis.2.IPI score,ECOG score,Ann Arbor stage,primary site,number of extranodal involvement,albumin,LDH,EBV,serous effusion associate with prognosis of NUAT-ENKTCL.LDH and severe complication are independent prognostic factor of NUAT-ENKTCL.3.There are no difference between surgery combined with chemotherapy and chemotherapy alone on NUAT-ENKTCL survival rates. |
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