Report of Consensus Panel 4 from the 12th International Workshop on Waldenstr&#246;m&#39;s Macroglobulinemia on the management of patients with non-IgM lymphoplasmacytic lymphoma

Tedeschi, A; Auer, R; Autore, F; Castillo, JJ; Gatt, ME; Kimby, E; Moreno, DF; Owen, RG; Qiu, L; Roccaro, AM; Sarosiek, S; Sekiguchi, N; Seymour, JF; Varettoni, M; Patterson, CJ; Matous, JV; Buske, C; Treon, SP; Sanz, RG

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

Author(s): Tedeschi, A; Auer, R; Autore, F; Castillo, JJ; Gatt, ME; Kimby, E; Moreno, DF; Owen, RG; Qiu, L; Roccaro, AM; Sarosiek, S; Sekiguchi, N; Seymour, JF; Varettoni, M; Patterson, CJ; Matous, JV; Buske, C; Treon, SP; Sanz, RG;
Details: Publication Year 2025-04-08,Volume 62,Issue #2,Page 106-112
Journal Title: Seminars in Hematology
Publication Type: Guideline
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.
Publisher: Elsevier
Keywords: Lymphoplasmacytic lymphoma; Myd88; Response assessment; Treatment
Department(s): Haematology
Publisher's Version: https://doi.org/10.1053/j.seminhematol.2025.04.002
Terms of Use/Rights Notice: Refer to copyright notice on published article.

Creation Date: 2025-07-17 04:27:26

Last Modified: 2025-07-17 04:28:05