The T-Cell Predominance: Angioimmunoblastic T-Cell Lymphoma

Journal of Cancer Research and Immuno-Oncology is an open access peer reviewed journal in the field of cancer research. Journal of Cancer Research and Immuno-Oncology covers a broad scope for the authors to make their valuable contributions in cancer biology, tumor, radiology, metastatis, cancer immunotherapy, oncology, radiation therapy etc

A lymph node based T-cell lymphoma which originates from a T follicular helper cell phenotype may cogitate the angio-immunoblastic T cell lymphoma (AITL) or an angio-immunoblastic lymphadenopathy with dysproteinaemia (AILD). At an estimated 1-2% of Non-Hodgkin’s lymphoma, it may emerge at a median age of 59-65 years with a slight male predominance. Approximately 70% individuals exemplify B symptoms such as fever, weight loss greater than 10% of the body weight, drenching night sweats, lymph node enlargement, hepato-splenomegaly (74%) and skin involvement (50%).

The immune hyper-active lymphoma may enunciate an elevation of the erythrocyte sedimentation rate (ESR), reactive autoimmune rheumatoid factor (RF), anti-smooth muscle antibody and coexistent circulating immune complexes or a cold agglutinin reaction. An all prevailing dys-regulation of the follicular T helper (TFH) lymphocytes ensues within the disorganized germinal centres with an emerging angio-immunoblastic T cell lymphoma.

Immunoblasts, B lymphocytes, plasma cells, eosinophils, histiocytes and epitheloid cells may predominate with diverse immune reactive T cell antigens such as CD3+, CD4+, CD8-, CXCL13+, CD10+, BCL6-, CD19+, C20+, CD1a+, CD21+, CD23+ and TdT. Multiple genetic aberrations such as TET2 47-73%, DN MT 3A (33%) and IDH2-R172 20-40% may be exemplified. The classic combination of cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) is a gold standard of therapy with AITL. Solitary agents employed in combination with CHOP regimen are romidepsin, belinostat or pralatrexate. Median 5year survival of the lymphoma (AITL) appears at an estimated (32%).

Cyclosporine as an immune suppressive agent is utilized in AITL with an objective response rate (ORR) of 75%. Immune-modulatory agent lenalidomide is appropriate for managing refractory or relapsed AITL. Achieved objective response rate (ORR) is at 29% with emergence of partial remission. Romidepsin and Belinostat are employed as FDA approved singular therapies for treating refractory or relapsed instances of AITL. However, the agents are not efficacious as front line therapy for treating refractory AITL in combination with CHOP. Novel therapies such as Janus Kinase (JAK 2) inhibitors, hypo-methylating agents and isocitrate dehyrogenase 2(IDH2) inhibitors prove to be efficacious in clinical trials.

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