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. 2025 Apr;62(2):106-112.
doi: 10.1053/j.seminhematol.2025.04.002. Epub 2025 Apr 8.

Report of Consensus Panel 4 from the 12th International Workshop on Waldenstrom's Macroglobulinemia on the management of patients with non-IgM lymphoplasmacytic lymphoma

Alessandra Tedeschi  1 , Rebecca Auer  2 , Francesco Autore  3 , Jorge J Castillo  4 , Moshe E Gatt  5 , Eva Kimby  6 , David F Moreno  7 , Roger G Owen  8 , Lugui Qiu  9 , Aldo M Roccaro  10 , Shayna Sarosiek  4 , Naohiro Sekiguchi  11 , John F Seymour  12 , Marzia Varettoni  13 , Christopher J Patterson  4 , Jeffrey V Matous  14 , Christian Buske  15 , Steven P Treon  4 , Ramon Garcia Sanz  16
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Report of Consensus Panel 4 from the 12th International Workshop on Waldenstrom's Macroglobulinemia on the management of patients with non-IgM lymphoplasmacytic lymphoma

Alessandra Tedeschi et al. Semin Hematol. 2025 Apr.

Abstract

Approximately 95% of lymphoplasmacytic lymphomas (LPL) are IgM secreting and are characterized as Waldenstrom Macroglobulinemia (WM). Conversely, non-IgM secreting LPL are rare. As part of the 12th International Workshop on WM (IWWM-12), a consensus panel of experts was tasked to develop recommendations for the management and response assessment of non-IgM LPL. The panel considered that in view of available molecular, pathological and clinical data, non-IgM LPL should be considered as a separate sub-entity of LPL. The panel further recommended that the IWWM-2 consensus criteria used for IgM LPL (WM) treatment initiation, should also be used for non-IgM LPL and be independent of IgG or IgA paraprotein level unless symptomatic hyperviscosity is present. The panel agreed that based on current evidence, there is insufficient data to support a different clinical management for non-IgM vs IgM (WM) LPL. Moreover, the panel advised that patients with non-IgM LPL should be treated in a similar manner to patients with IgM LPL independent of MYD88 mutation status until more is known about its impact on treatment outcomes for non-IgM LPL patients. The panel therefore recommends the use of the IWWM-11 IgM LPL (WM) response criteria for cases of non-IgM LPL with a monoclonal IgA or IgG paraprotein component, but creating a specific panel to develop formal response criteria for this LPL subset was also recommended.

Keywords: Lymphoplasmacytic lymphoma; MYD88; Response assessment; Treatment.

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Conflict of interest statement

Declaration of competing interest The authors declare the following financial interests/personal relationships which may be considered as potential competing interests: Alessandra Tedeschi Received honoraria from AbbVie, J&J, Beigene, Lilly, Astrazeneca Rebecca Auer Received clinical trial funding received from Janssen Pharmaceuticals, travel / conference support from Abbvie, honoraria from Astra Zeneca and Beigene. Francesco Autore Received honoraria from Abbvie, AstraZeneca, Beigene, and Johnson&Johnson. Jorge J. Castillo Received honoraria from Abbvie, AstraZeneca, Beigene, Cellectar, Johnson & Johnson, Kite, Loxo, and Pharmacyclics, and research funds from Abbvie, AstraZeneca, Beigene, Cellectar, Loxo, and Pharmacyclics. Moshe Gatt No disclosures. Eva Kimby Received Reports consulting fees from Abbvie, Beigene, Eli-Lilly, Pierre Fabre, Janssen, Pierre Fabre, Celgene, honoraria for educational lectures: Astra Zeneca, Beigene, Abbvie, Abbvie, Janssen, and participation on a DSMB for Genmab. David F. Moreno Received travel grants and honoraria from Janssen. Roger Owen Consulting and Speaker’s Bureau for Beigene. Lugui Qiu Received research funding, and/or consulting fees from Beigene, AbbVie, Johnson & Johnson, Sanofi, Roche. Aldo Roccaro Received research funding from International Waldenstrom's Macroglobulinemia and the Luekemia and Lymphoma Society; Transcan2-ERANET; AstraZeneca; Associazione Italiana contro Leucemie, Linfomi e Mieloma (AIL) Brescia; Associazione Italiana per la Ricerca sul Cancro (AIRC); European Hematology Association (EHA); and honoraria from Abbvie; AstraZeneca; Beigene; Daiichi-Sankyo; Janssen; Takeda. Shayna Sarosiek Received research funding and/or consulting fees from BeiGene, Cellectar, and ADC Therapeutics. Naohiro Sekiguchi Received honoraria from Janssen, Ono, BeiGene and research funding from Incyte Biosciences Japan, Jans¬sen, Mitsubishi Tanabe Pharma Corporation, MSD, Ono. John F. Seymour Consultancy for Genor Bio, TG Therapeutics; Research Funding from Abbvie, BMS, Roche. Speakers bureau for AbbVie, Astra Zeneca, BMS, Roche; speaking honoraria from AbbVie, Astra Zeneca, Beigene, BMS, Gilead, Janssen, Roche. Marzia Varettoni Received research funding, and/or consulting fees from AbbVie, Astrazeneca, BeiGene, Janssen Christopher J. Patterson No disclosures. Jeffrey V. Matous Received consulting fees from Beigene. Christian Buske Reports consultancy, honoraria, advisory board, and travel expenses from Roche/Genentech, Janssen, BeiGene, Novartis, Pfizer, Incyte, AbbVie, Gilead, Celltrion, MorphoSys, Regeneron, Sobi, and Lilly. Steven P. Treon Received research funding and/or consulting fees from Abbvie/Pharmacyclics, Janssen, Beigene, Lilly, BMS, and Ono Pharmaceuticals. Ramon Garcia Sanz Received research funding, and/or consulting fees from Gilead, Astellas, Takeda, Johnson & Johnson, BeiGene.

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