Approach to T-Follicular Helper (TFH) Lymphomas and Mimics
Follicular helper T-cell (TFH) lymphomas comprise a unique group of T-cell lymphomas that represent neoplastic proliferations of follicular helper T-cells and share genetic, immunophenotypic, morphologic, and clinical features. Angioimmunoblastic T-cell lymphoma (AITL) is the prototypical TFH lymphoma; in addition, the 5th World Health Organization (WHO) and the International Consensus Classification (ICC) recognizes two other unique subtypes: follicular type and not otherwise specified (NOS). TFH lymphomagenesis is largely driven by the stepwise acquisition of somatic mutations with TET2 and DNMT3A mutations occurring early in hematopoietic stem cells. Subsequently, other characteristic mutations such as RHOA and IDH2 (in AITL) are observed. This presentation discusses the morphologic spectrum, immunophenotype, diagnostic mimics/pitfalls, and unique genetic attributes of this category of T-cell lymphomas.
