On March 10–12 2017, the EHA-SWG meeting on Rare Lymphomas took place in Barcelona, Spain, and was jointly chaired by Prof. Martin Dreyling, from Klinikum der Universität München, Germany, and Prof. Marie José Kersten, from the Academic Medical Center, Amsterdam, The Netherlands.
Firstly, an overview of the learning objectives was given:
Challenges in PTCLs include: great clinicopathological heterogeneity, difficult diagnosis, cell of origin, oncogenic events still poorly characterized, rare diseases (geographic variations), and rarity of in vitro and in vivo models. It was stated that there is an unmet medical need: 1/3 are primary refractory, 1/3 are relapses, and it is uncertain if the remaining 1/3 are cured long-term.
It was also stated that PTCL cell of origin is T helper subsets:
An overview of the 2016 WHO classification of PTCL was also given:
In terms of PTCL-NOS, it was stated that every case is a “diagnosis of exclusion”; an extensive panel of IHC markers (TFH markers, FDC, EBV, CD30, cytotoxic, etc.) to exclude a better defined PTCL entity, in particular PTCL of TFH origin, ALCL, or involvement by an extranodal PTCL.
To finish the talk, P. Gaulard summarized the conclusions and challenges of PTCLs: