Clinical Characteristics And Prognosis In 84 Patients With Angioimmunoblastic T-cell Lymphoma

Background and objective Angioimmunoblastic T-cell lymphoma(AITL)is one of the clinically rare and highly malignant peripheral T-cell lymphoma(PTCL)subtypes,which approximately accounts for 18.5% all T-cell and NK-cell lymphomas.With the continuous improvement of medical care and patients’ medical awareness,the diagnosis of AITL continues to increase,but there are still some atypical AITL,increasing the difficulty in diagnosis and treatment.In order to clarify the clinical characteristics and prognostic factors of AITL,we conducted a retrospective study of AITL patients from Fujian Medical University Union Hospital to provide a basis for early diagnosis and early treatment of AITL.Methods The clinical data of 84 patients(including 10 patients who were not treated in our hospital)with AITL,admitted to the Fujian Medical University Union Hospital from July,2009 to September,2018,were collected and the clinical characteristics and prognostic factors were retrospectively analyzed.The diagnosis of AITL was based on criteria established by the World Health Organization criteria(WHO 2008 and 2016).The clinical stage was based on the Ann Arbor criteria,the physical performance status(PS)was divided into 1 to 5 according to the Eastern Cooperative Oncology Group(ECOG),and the risk stratification was assigned according to international prognostic index(IPI)and prognostic index for peripheral T-cell lymphoma-not otherwise specified scores(PIT).Statistical software used SPSS 22.0.Univariate and multivariate analyses were performed with Kaplan-Meier method,Long-rank test and Cox proportional hazards regression models,P <0.05 was statistically significant.Results The median age of 84 patients was 62 years(range,39-86 years)with 52.4% of patients older than 60 years.The male-to-female ratio was 2.5:1.Almost all patients(80/86;95.2%)presented with advanced-stage disease(stage III-IV according to Ann Arbor classification)and B symptoms were observed in 53 cases(63.1%).Extranodal disease was present in 43 cases(51.2%)and bone marrow infiltration was found in 16cases(19.1%).An ECOG performance status of ≥2 was observed in 18 cases(21.4%).Laboratory examination showed that 7 patients(8.3%)had white blood cell count <4*10^9/L,51 cases(60.7%)had normal white blood cell count(4-10*10^9/L)and others(26 cases,31.0%)had increased white blood cell count(>10*10^9/L).Anemia(hemoglobin <110g/L)and hypoalbuminemia were seen in 39(46.4%)and 62cases(73.8%),respectively.The presence of elevated serum globulin,LDH,and Beta2-microglobulin level were noted in 39(46.4%),62(73.8%)and 39 cases(46.4%),respectively.CT scan revealed that 48 patients(57.1%)had serous effusions.Bone marrow in situ hybridization showed that 35 cases(41.7%)had EBER-positive and 28cases(33.3%)had EBER-negative.59 cases(70.2%)were with intermediate high and high risk according to international prognostic index and 42 cases(50.0%)were with intermediate high risk according to prognostic index for peripheral T cell lymphoma.Of the 84 patients,67 patients received treatment in our hospital after diagnosis,including chemotherapy(61 cases were treated with CHOP or CHOP-like,and 6 cases received other chemotherapy regimens),autologous hematopoietic stem cell transplantation(6cases),allogeneic hematopoietic stem cell transplantation(2 cases,one of which underwent autologous hematopoietic stem cell transplantation and allogeneic hematopoietic stem cell transplantation)and so on.Seven patients did not received chemotherapy and died due to rapid disease progression or other serious complications,the other 10 patients were not treated in our hospital after diagnosis.The treatment response was evaluable in 61 of 67 patients who received chemotherapy.The complete remission(CR/CRu)rate was 26.2%(16 of 61),and the partial response(PR)rate was41.0 %(25 of 61).The median overall survival(OS)and progression-free survival(PFS)in 61 patients was 23 and 14 months,and the 1-year to 5-year OS rates were 66.6,61.0,53.3,50.2 and 46.0%,respectively.Fifteen(36.6%)of 41 patients who achieved CR or PR experienced relapse.17 patients who did not receive chemotherapy in our hospital died except for 3 cases who were lost to follow-up.On univariate analysis,age,IPI score,PIT score,hemoglobin level,presence of serous effusions,and chemotherapy influenced OS.Age >60 years,hemoglobin <110g/L and presence of serous effusions were poor prognostic factors for PFS.Multivariate analysis revealed that age >60 years and serous effusion were independent poor prognostic factors for OS,and age >60 years is an significant prognostic factors for PFS.Conclusion AITL is a Non-Hodgkin lymphomas with high invasion,rapid progress and poor prognosis.The response rate of first-line treatment is high,while the recurrence rate is also high,And the age,IPI score,PIT score,hemoglobin levels and serous effusion can be used as reference factors for judging the prognosis of AITL.