Abstract: To retrospectively analyze patients with angioimmunoblastic T cell lymphoma ( AITL ) from a single institution, and to define the clinical features and effect of chemotherapy. Methods: Up to 23 patients with AITL in our department from Sept.1999 to Sept. 2010 were enrolled, but only 21 patients received treatment. Up to 17 patients received CHOP-1ike regimens as initial chemotherapy treatments, 4 patients received L-asparaginase ( L-asp ) + bleomycin + dexamethasone + vindesine regimen; 1 patient was treated with radiotherapy in every group and 1 patient with high-dose chemotherapy followed by autologous stem cell transplantation ( HDT-ASCT ) in every group as upfront consolidation therapy. Salvage regimen was ICE, DHAP or ProMACE/CytaBOM. Five patients received interferon or combined thalidomide for maintenance therapy; 3 patients received chidamide. Results: The median age of the 23 patients was 60 years; the male to female ratio was 1.9:1. Among all patients, 96% were in Ann Arbor stage Ⅲ or Ⅳ, 57% presented with B symptoms, 22% had splenic involvement or splenomegaly. In terms of laboratory features, 39% had elevated lactate dehydrogenase ( LDH ), 75% had elevated beta-2 microglobulin ( β2-MG ), and D-dimer levels exceeding 255 ng/mL were observed in 80% patients at diagnosis. Immunohistochemistry indicated that the positive rate for CXCL13 was 100% ( 12/12 ) and EBER was 80% ( 8/10 ). Analysis of the therapeutic effect revealed 7 patients achieved complete remission ( CR ) and 8 patients achieved partial remission ( PR ) after initial treatment with CHOP-1ike regimens; 1 patient achieved CR and 1 patient achieved PR after initial treatment with L-asp-based regimens. The median overall survival ( OS ) was 27 ( 2.9-51.1 ) months, whereas the 3- and 5-year OS was 44% and 29%. Up to 90% of the patients suffered from bone marrow depression Ⅲ or Ⅳ, 33% patients suffered from pneumonia, and 1 patient suffered herpes zoster after chemotherapy. Conclusion: AITL is a distinct subtype of peripheral T-cell lymphoma with unique clinical and pathologic features. In our study, most of the patients had abnormal clotting mechanisms. AITL patients often exhibit immunodeficiency; thus, bone marrow depression and opportunistic infections during therapy cannot be neglected.