Entities versus diseases: Myers et al. propose distinct aspects of adult T-cell lymphoma/leukemia
Year: 2024
Type: article
Abstract: In this issue of Haematologica, Myers et al. 1 argue that the characteristics of adult T-cell lymphoma/leukemia (ATLL) differ based on the geographic location of the patients affected.ATLL is a well-established entity, whose diagnostic criteria have remained substantially unchanged in the International Consensus Classification 2 and 5 th Edition of the WHO Classification of Haemato-lymphoid Tumours 3 as compared to those in the 2017 WHO Classification.4 Although ATLL is recorded in different geographic areas, all in which the retrovirus human T-lymphotropic virus type 1 (HTLV-1) is endemic, most information in the literature is based on Japanese cases.Accordingly, the prototypic description of ATLL includes four subtypes (acute, lymphomatous, chronic, and smoldering), with different clinical characteristics, aggressiveness, cytological atypia, and prognosis.The average age of patients is 58 years.On molecular grounds, Japanese cases show frequent gain-of-function in T-cell receptor/NF-kB signaling, including activating mutations of PLCG1 and PRKCB, CTLA4/ICOS-CD28 fusions, and REL truncations.5 Recurrent alterations of the CD274 locus correlate with PD-L1 overexpression.5 Further abnormalities affect transcriptional regulation, T-cell trafficking (CCR4 and CCR7 truncating mutations), tumor suppression (TP53) and epigenetic modification (ARID2 and EP300).5 Aggressive subtypes are characterized by more genetic alterations, while indolent subtypes frequently carry STAT3 mutations.5 Patients with CCR4 mutations may respond to mogamulizumab, 5 while those with single nucleotide variations and copy number alterations of TP53 have an inferior overall survival, regardless of the type of treatment.5 The paper by Myers et al. sheds new light on the topic of ATLL. 1 It focuses on the characteristics of cases occurring in Western populations and compares them with those of Japanese cohorts.One-hundred sixty-five ATLL cases were collected in South American and Caribbean countries as well as in France and USA, the latter corresponding to patients of African descent.Such a cohort has never been
Source: Haematologica
Author: Stefano A. Pileri
Institutions: European Institute of Oncology, University of Bologna
Language: English
Subfield: Immunology
Field: Immunology and Microbiology
Domain: Life Sciences
Open Access status: gold
Estimated APC paid: $2,157
OpenAlex ID: W4402015432