Page 15 - Haematologica - Vol. 105 n. 6 - June 2020
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 Editorials
     Figure 1. Peripheral T-cell lymphoma classification following the World Health Organization 2017 Lymphoma Classification, here restricted to the tumor types covered in the article. PTCL: Peripheral T-cell Lymphoma. NK: Natural Killer; ATLL: Adult T-cell Leukemia/Lymphoma; NOS: Not otherwise specified; TFH: T-Follicular Helper; ALK: Anaplastic Lymphoma Kinase; TH: T Helper; AITL: Angioimmunoblastic T-cell Lymphoma.
 with the cases divided among the different phenotypes. In this study, ALK-negative ALCL had two distinct profiles, with or without expression of cytotoxic genes.
Clinical correlation confirmed the poor prognosis of PTCL (5-year OS=27%), and the better prognosis for ALK+ and DUSP22+ ALCL, but failed to recognize markers that recognize groups with additional clinical variability. In particular, the division into TH1 and TH2 phenotypes in PTCL-NOS was not found to be clinically significant. It is of particular note that a 90% concordance was obtained between the three centers, thus emphasizing one of the stronger points in this approach, i.e. better reproducibility.
Although this is almost certainly not the last word on the process of creating molecular tools for the routine diagnosis of PTCL, it is an important step forward that establishes the viability of the proposal to integrate gene expression and variant recognition, and raises some ques- tions about the PTCL subclasses as they are currently rec- ognized.
Peripheral T-cell lymphoma diagnosis is an area in which molecular diagnosis can play an important role in the near future. Efforts in this field could prove even more relevant, with the provision, not only of diagnostic mark- ers, but also of predictive therapeutic markers, by which the different lymphoma categories may be associated
with the identification of useful targets that can be exploited for therapeutic purposes.
Funding
This work was supported by grants from the Instituto de Salud Carlos III (ISCIII) of the Spanish Ministry of Economy and Competence (MINECO, RTICC ISCIII and CIBERONC) (SAF2013-47416-R, RD06/0020/0107-RD012/0036/0060 and Plan Nacional I+D+I: PI17/2172, PI16/01294 and PIE15/0081), AECC, and the Madrid Autonomous Community.
References
1. Mak V, Hamm J, Chhanabhai M, et al. Survival of patients with peripheral T-cell lymphoma after first relapse or progression: spec- trum of disease and rare long-term survivors. J Clin Oncol. 2013;31(16):1970-1976.
2. Federico M, Bellei M, Marcheselli L, et al. Peripheral T cell lym- phoma, not otherwise specified (PTCL-NOS). A new prognostic model developed by the International T cell Project Network. Br J Haematol. 2018;181(6):760-769.
3. Weisenburger DD, Savage KJ, Harris NL, et al. Peripheral T-cell lym- phoma, not otherwise specified: a report of 340 cases from the International Peripheral T-cell Lymphoma Project. Blood. 2011;117(12):3402-3408.
4. Laurent C, Baron M, Amara N, et al. Impact of Expert Pathologic Review of Lymphoma Diagnosis: Study of Patients From the French Lymphopath Network. J Clin Oncol. 2017;35(18):2008-2017.
5. deLevalL,RickmanDS,ThielenC,etal.Thegeneexpressionprofile
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