Angioimmunoblastic T-cell Lymphoma:4 Cases Report And Literature Review

Background and Objective: Angioimmunoblastic T-cell lymphoma(AITL)is a subtype of peripheral T-cell lymphoma,which is prone to clinical misdiagnosis due to its complex histopathology and immunophenotype.At present,the efficacy of CHOP and E-CHOP chemotherapy is uneven in AITL.Only a few patients get complete remission through chemotherapy,but they are prone to relapse.Most patients have poor efficacy of chemotherapy,which is full of challenges for refractory and recurrent patients.With the increase of the incidence of the disease in recent years,it is urgent for us to conduct in-depth research on the pathogenesis,clinicopathological features and treatment progress of the disease in order to improve the comprehensive ability of its clinical diagnosis and treatment.Try to achieve early diagnosis and early treatment,and avoid misdiagnosis and mistreatment.In order to improve the understanding of this disease,we analyzed the data we got.Methods:To collect the cases which was diagnosed with AITL in my hospital from August 2017 to March 2020,All cases should have complete pathological findings.Four cases were selected.The clinical data we selected were retrospectively analyzed,summarized at home and abroad and to retrieve relevant literature,looking for the common in clinical manifestation,immunohistochemical and the treatment outcome.Results: Among the 4 cases,the average age is 60 years old,and The ratio of men to women is equal.Clinical manifestations: 2 patients had fever and systemic rash in the course of disease;All 4 patients had enlarged lymph nodes on both sides of the transverse septum,among which 1 patient was found to have generalized lymph node enlargement indirectly due to other diseases.Imaging examination: all 4 patients had splenomegaly,but none had hepatomegaly.Three patients developed pleural effusion during the course of the disease,two of which had suspicious pulmonary infiltrating changes.Laboratory examination: 2 cases had mild anemia.The hemolysis test was negative in 4 patients and positive in 2patients.Lactate dehydrogenase was significantly elevated in 1 case and beta-2-microglobulin in 2 cases.Lymph node tissue features: lymph node destruction,follicular dendritic cells,small vascular hyperplasia,inflammatory cell infiltration.Immunological phenotype: all the 4 patients had the expression of T-cell common antigen,Follicular helper T-cell antigen,B-cell antigen and tumor-specific markers.Treatment and prognosis: mainly adopts E-CHPO solution chemotherapy,among them 2 patients without progression,1 patients with poor curative effect was finally given the Hyper-CVAD scheme and joint immunosuppressant,At this time her fever and rash were controled,but her bone marrow and lung were involved by tumour when checked,her disease continued to progress,short-term appeared suspicious lung infiltrates,and then she died due to the complications of lung infection.Although one patient had pulmonary infiltration in a short time,his disease was controled after chemotherapy.Conclusion: 1.The clinical incidence of AITL is low.Fever,rash,hepatosplenomegaly and lymphadenomegaly are the main clinical manifestations of AITL,but not all patients have them at the same time.2.Early lymphadenopathy is easy to be ignored and easily confused with other lymphadenopathy and other related diseases.2.The background cells of AITL pathological tissue are complex,with more inflammatory cells and diversified immune manifestations,which are easy to be misdiagnosed.Some patients even need multiple pathological examinations to make a diagnosis.3.There is no unified standard of treatment for AITL patients.At present,the treatment is CHOP,E-CHOP or similar CHOP chemotherapy.Patients eligible for transplantation can receive large doses of consolidation therapy and autologous or allogeneic stem cell transplantation.New targeted therapy drugs are emerging,and chemotherapy drugs combined with targeted drug therapy has gradually become the trend of treatment.4.Angioimmunoblastic T-cell lymphoma has a poor prognosis and high mortality,so the study of molecular genetics may provide ideas for the development of new drugs.