がん研究と免疫腫瘍学ジャーナル
オープンアクセス

概要

The T-Cell Predominance: Angioimmunoblastic T-Cell Lymphoma

Anubha Bajaj

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 5 year survival of the lymphoma (AITL) appears at an estimated (32%).