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Classification and diagnostic evaluation of nodal T- and NK-cell lymphomas

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Abstract 

Nodal T- and NK-cell lymphomas are among the most frequent T-cell malignancies and most subtypes have aggressive clinical behavior. Evolving understanding of the biology and molecular characteristics of these lymphomas, as well as the development of new precision therapy approaches, underscores the importance of ongoing updates to the classification and diagnostic evaluation of this group of malignancies. Here, we discuss the classification of nodal T- and NK-cell lymphomas based on the 2022 International Consensus Classification of Mature Lymphoid Neoplasms (2022 ICC). Lymphomas of T-follicular helper cell origin are now grouped into a single entity, follicular helper T-cell lymphoma (TFH lymphoma), with three subtypes (angioimmunoblastic-type, follicular-type, and not otherwise specified), reflecting their common cellular origin and shared molecular and clinical characteristics. Classification of anaplastic large cell lymphoma (ALCL) remains essentially unchanged; DUSP22-rearranged cases are now considered a genetic subtype of ALK-negative ALCL. Primary nodal EBV-positive T-/NK-cell lymphoma is introduced as a new provisional entity; these cases were previously considered a variant of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS). PTCL, NOS remains a diagnosis of exclusion, with evolving molecular data indicating the presence of distinct subgroups, including PTCL-TBX21, PTCL-GATA3, and EBV-negative cytotoxic PTCLs. We also discuss diagnostic strategies to facilitate the 2022 ICC classification among nodal T- and NK-cell lymphomas and the distinction from nodal involvement by extranodal neoplasms.

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Authors and Affiliations

  1. Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA

    Andrew L. Feldman

  2. Department of Pathology, IUCT-Oncopole, and Laboratoire d’Excellence TOUCAN, Toulouse, France

    Camille Laurent

  3. Department of Pathology, Instituto de Investigaciones Hematológicas, Academia Nacional de Medicina, Buenos Aires, Argentina

    Marina Narbaitz

  4. Department of Pathology and Laboratory Medicine, Nagoya University Hospital, Nagoya, Japan

    Shigeo Nakamura

  5. Department of Pathology, City of Hope National Medical Center, Duarte, CA, USA

    Wing C. Chan

  6. Institute of Pathology, Department of Laboratory Medicine and Pathology, Lausanne University Hospital and Lausanne University, Lausanne, Switzerland

    Laurence de Leval

  7. Department of Pathology, University Hospital Henri Mondor and University Paris Est, Créteil, France

    Philippe Gaulard

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  1. Andrew L. Feldman
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  2. Camille Laurent
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  3. Marina Narbaitz
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Contributions

ALF, CL, MN, SN, WCC, LdL, and PG are members of the Clinical Advisory Committee that prepared the 2022 ICC classification of lymphoid neoplasms. ALF drafted the manuscript. CL, MN, SN, WCC, LdL, and PG reviewed and corrected the manuscript.

The authors declare that they have followed the principles of ethical and professional conduct.

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Correspondence to Andrew L. Feldman.

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Feldman, A.L., Laurent, C., Narbaitz, M. et al. Classification and diagnostic evaluation of nodal T- and NK-cell lymphomas. Virchows Arch 482, 265–279 (2023). https://doi.org/10.1007/s00428-022-03412-6

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