Angioimmunoblastic T-Cell Lymphoma: Diagnostic Pathology and Molecular Landscape
This review details the multistep pathogenesis of AITL driven by sequential mutations in TET2, DNMT3A, RHOA, and IDH2, derived from T-follicular helper cells. For diagnostic labs, the characteristic mutation profile and immunophenotype (PD-1, CXCL13, ICOS expression) enable molecular and immunohistochemical distinction from other T-cell lymphomas, guiding appropriate targeted therapy selection.
The original study
Angioimmunoblastic T-cell lymphoma: Current Diagnostic Insights and Advances.
- Authors
- Matsumoto NP, Xu ML
- Journal
- Human pathology
- Type
- Journal Article, Review, Case Reports
- PMID
- 39571692
Original abstract
Angioimmunoblastic T-cell lymphoma (AITL), or nodal T-follicular helper cell lymphoma, angioimmunoblastic type, is a rare and aggressive type of T-cell lymphoma characterized by a spectrum of clinical and histopathological features that can present diagnostic challenges. Derived from T-follicular helper cells, the genesis of AITL is thought to be a multistep process involving mutations in epigenetic regulatory genes such as TET2 and DNMT3A, followed by driver mutations in RHOAG17V and IDH2R172 which promote clonal expansion as well as a characteristic inflammatory milieu. This review aims to provide a comprehensive overview of AITL, including its clinical presentation, epidemiology, pathogenesis, histomorphology and treatment options. Despite advancements in the understanding of AITL biology and the development of novel treatment strategies, the prognosis for patients with AITL remains poor.