Angioimmunoblastic T-Cell Lymphoma (AITL)

Overview

Angioimmunoblastic T-Cell Lymphoma is a Mature T and NK Neoplasm (parent MTNN) and a subtype of peripheral T-cell lymphoma.

Cohorts in the corpus

Recurrent alterations

  • RHOA: 88% of RHOA mutations in AITL were the canonical G17V variant PMID:37078708.
  • TET2, DNMT3A, IDH2: frequently mutated across the PTCL cohort that is 47% AITL PMID:37078708.
  • TP53 mutations and 17p deletions were the only somatic aberrancies independently correlated with inferior PFS on CHOP-based therapy in the PTCL cohort PMID:37078708.
  • RHOA G17V dominant-negative mutation is present in ~67% of AITL cases, abolishes GTP binding, and is functionally characterised as loss-of-function in a yeast complementation model PMID:24816253

Subtypes

Therapeutic landscape

  • Curative-intent CHOP-based chemotherapy (CHOP, CHOEP/EPOCH, BV-CH(E)P) is the standard first-line approach in this cohort PMID:37078708.

Sources

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