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

IF 4.1 3区 医学 Q1 HEMATOLOGY
Alessandra Tedeschi , Rebecca Auer , Francesco Autore , Jorge J. Castillo , Moshe E. Gatt , Eva Kimby , David F. Moreno , Roger G. Owen , Lugui Qiu , Aldo M. Roccaro , Shayna Sarosiek , Naohiro Sekiguchi , John F. Seymour , Marzia Varettoni , Christopher J. Patterson , Jeffrey V. Matous , Christian Buske , Steven P. Treon , Ramon Garcia Sanz
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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.
第12届Waldenström巨球蛋白血症国际研讨会关于非igm淋巴浆细胞性淋巴瘤患者管理的共识小组报告
大约95%的淋巴浆细胞性淋巴瘤(LPL)分泌IgM,其特征为Waldenstrom巨球蛋白血症(WM)。相反,非igm分泌的LPL是罕见的。作为第12届WM国际研讨会(iwm -12)的一部分,一个协商一致的专家小组的任务是为非igm LPL的管理和反应评估提出建议。专家组认为,鉴于现有的分子、病理和临床数据,非igm LPL应被视为LPL的一个单独的子实体。专家组进一步建议,用于IgM LPL (WM)治疗起始的iwm -2共识标准也应用于非IgM LPL,并且不依赖于IgG或IgA副蛋白水平,除非出现症状性高粘稠度。专家组一致认为,根据目前的证据,没有足够的数据支持非IgM与IgM (WM) LPL的不同临床管理。此外,专家组建议,非IgM LPL患者应采用与独立于MYD88突变状态的IgM LPL患者相似的治疗方式,直到更多地了解其对非IgM LPL患者治疗结果的影响。因此,专家组建议对含有单克隆IgA或IgG副蛋白成分的非IgM LPL病例使用iwm -11 IgM LPL (WM)反应标准,但也建议为该LPL子集创建一个专门的专家组来制定正式的反应标准。
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来源期刊
Seminars in hematology
Seminars in hematology 医学-血液学
CiteScore
6.20
自引率
2.80%
发文量
30
审稿时长
35 days
期刊介绍: Seminars in Hematology aims to present subjects of current importance in clinical hematology, including related areas of oncology, hematopathology, and blood banking. The journal''s unique issue structure allows for a multi-faceted overview of a single topic via a curated selection of review articles, while also offering a variety of articles that present dynamic and front-line material immediately influencing the field. Seminars in Hematology is devoted to making the important and current work accessible, comprehensible, and valuable to the practicing physician, young investigator, clinical practitioners, and internists/paediatricians with strong interests in blood diseases. Seminars in Hematology publishes original research, reviews, short communications and mini- reviews.
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