https://orcid.org/0000-0003-3994-516X
Abstract
T- and natural killer (NK)-cell lymphomas are neoplasms derived from immature T cells (lymphoblastic lymphomas), or more commonly, from mature T and NK cells (peripheral T-cell lymphomas, PTCLs). PTCLs are rare but show marked biological and clinical diversity. They are usually aggressive and may present in lymph nodes, blood, bone marrow, or other organs. More than 30 T/NK-cell–derived neoplastic entities are recognized in the International Consensus Classification and the classification of the World Health Organization (fifth edition), both published in 2022, which integrate the most recent knowledge in hematology, immunology, pathology, and genetics. In both proposals, disease definition aims to integrate clinical features, etiology, implied cell of origin, morphology, phenotype, and genetic features into biologically and clinically relevant clinicopathologic entities. Cell derivation from innate immune cells or specific functional subsets of CD4+ T cells such as follicular helper T cells is a major determinant delineating entities. Accurate diagnosis of T/NK-cell lymphoma is essential for clinical management and mostly relies on tissue biopsies. Because the histological presentation may be heterogeneous and overlaps with that of many benign lymphoid proliferations and B-cell lymphomas, the diagnosis is often challenging. Disease location, morphology, and immunophenotyping remain the main features guiding the diagnosis, often complemented by genetic analysis including clonality and high-throughput sequencing mutational studies. This review provides a comprehensive overview of the classification and diagnosis of T-cell lymphoma in the context of current concepts and scientific knowledge.
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Comments
Universal HTLV-1 Serology in T-Cell Lymphoma Diagnostic Workup
Omitting HTLV-1 testing in patients from non-endemic areas with a diagnosis of T-cell lymphoma, as the authors suggest, may perpetuate a concerning cycle: without testing, ATLL will remain undiagnosed; this oversight can reinforce the misconception that ATLL does not occur in non-endemic regions; this, in turn perpetuates the belief that HTLV-1 serology is unnecessary. This situation is particularly troubling since ATLL can clinically and pathologically mimic other T-cell lymphomas(2), yet requires distinctly different therapeutic strategies, especially regarding the need for antiviral treatment and the indication for allogeneic bone marrow transplantation(3).
HTLV-1 serology is a quick, non-invasive and inexpensive test that can be incorporated in the regular workup of patients diagnosed with T-cell lymphomas without adding morbidity. It could reduce potential ATLL underdiagnosis, ensuring that all patients receive the most appropriate treatment. For these reasons, even in a scenario of low HTLV-1 prevalence, I believe that testing all patients with a diagnosis of T-cell lymphoma should be recommended.
Conflict of Interest: The author has no conflicts of interest related to the subject matter to declare.
Author contact: ygonzaga@inca.gov.br
References
1. de Leval LL, Gaulard P, Dogan A. A practical approach to the modern diagnosis and classification of T-and NK-cell lymphomas. Blood. May 10 2024;doi:10.1182/blood.2023021786
2. Khanlari M, Ramos JC, Sanchez SP, et al. Adult T-cell leukemia/lymphoma can be indistinguishable from other more common T-cell lymphomas. The University of Miami experience with a large cohort of cases. Mod Pathol. Jul 2018;31(7):1046-1063. doi:10.1038/s41379-018-0037-3
3. Katsuya H. Current and emerging therapeutic strategies in adult T-cell leukemia-lymphoma. Int J Hematol. Apr 2023;117(4):512-522. doi:10.1007/s12185-023-03572-4