Clinical Characteristics Of Mature Peripheral T-cell Lymphoma And Significance Of TCR/IgH Expressed By PTCL

Background and objective:Mature Peripheral T-cell Lymphomas(PTCL)are one group of rare and clinically divers neoplasms that represent approximately 12%of all non-Hodgkin's lymphomas(NHL).Incidences are higher in Asian population,represent 20%～35%of all NHL.PTCL are heterogeneous and their clinical characteristics,pathologic appearance and immunophenotype may vary within a given subtype.It is the rarity of these lymphomas that has resulted in a lack of understanding of their pathogenesis relative to the B-cell lymphomas and a dearth of the controlled clinical trials necessary to identify better approaches to therapy. Another clinical factors of PTCL impeding progress in therapy include relative chemoresistance and absence of a common marker for monoclonal antibody therapy.The prognosis in most types of PTCL is poor,with 5-survival less than 30%.Therapeutic advanced halve been slow,there is no consensus about optimal therapy in PTCL.The aim of this study was to analyses the clinical Characteristics and prognosis of PTCL,evaluate the significance of clonal gene rearrangements of immunoglobulin heavy chain(IgH)and T-cell receptorγ(TCRγ)in T-cell lymphomas.Methods:1.425 patients with PTCL treated from January 2000 to October 2006 in Zhejiang Cancer Hospital and The First Affiliated Hospital,Zhejiang University School of Medicine were enrolled and analysed retrospectively.2.IgH and TCRγgene rearrangements were studied with polymerase chain reaction(PCR)in 41 cases of PTCL.Bone marrow samples were taken from those patients.Results:1.Peripheral T-cell lymphomas represent 28.1%of all NHL. Peripheral T-cell lymphomas,unspecified(PTCL/U),NK/T cell lymphoma,nasal type,anaplastic large cell lymphoma(ALCL) were the more common subtypes.70.6%of patients wereâ…¢/â…£stage.48.6%of them had bone marrow involvement.The International Prognostic Index(IPI)score 2～4 was 65.6%.41.4% of them had B symptoms.2.Clonal gene rearrangements of IgH and/or TCRγwere found in 70.7%of PTCL.Clonal IgH gene rearrangements was detected in 43.9%of PTCL.TCRγgene rearrangements was detected in 46.3%of PTCL.3.2 and 5 year overall survival rates were 34%and 15%respectively. IPI score,hepatosplenomegaly,elevated lactate dehydrogenase (LDH),elevatedβ₂-Microglobulin were the significant prognostic factors of survival in Log rank method test,but age,involvement of Bone marrow,clonal gene rearrangements of IgH and/or TCRγ,adenopathy and different chemotherapy regiment were not.4.81.5%of Patients had NK/T cell lymphoma in Zhejiang Cancer Hospital were treated by radiotherapy plus chemotherapy.2 and 5 year overall survival rates of them were 43.3%å’Œ28.6% respectively.Conclusions:1.Peripheral T-cell lymphomas represented 28.1%of all NHL.Incidences were higher in China compared to Western population.In China,Extranodal NK/T cell lymphoma,the nasal type was more common.Majority of PTCL Patients were advanced.2.Clonal gene rearrangements of IgH and/or TCRγwere found in a majority of PTCL.3.The Prognosis of PTCL was poor.IPI was a valuable tool in the assessment of patients with PTCL. 4.CHOP chemotherapy was not the standard therapy.New approaches were warranted.Specific entities of PTCL need disease-adapted strategies,particularly for NK/T cell lymphoma...