Waldenstrom macroglobulinemia (WM)是一种独特的CD20+、B细胞非霍奇金淋巴瘤，其特点是骨髓淋巴浆细胞浸润和循环单克隆免疫球蛋白M。患者的临床表现和预后变异性很大。高水平证据支持将单克隆抗CD20抗体利妥昔单抗与化疗骨架相结合治疗WM。然而，随着对病理生理学的深入了解和布鲁顿酪氨酸激酶（BTK）抑制剂的纳入治疗范式，其当代管理变得更加微妙。了解患者的MYD88L265P和CXCR4突变状态可能有助于治疗决策。目前，两种常用的方法包括固定疗程化疗免疫疗法和基于BTK抑制剂的连续治疗直至进展。缺乏比较这两种截然不同方法的随机试验。最新研究表明，在以前暴露于共价BTK抑制剂的患者中，非共价BTK抑制剂和B细胞淋巴瘤-2（BCL2）抑制剂的疗效，证明了对WM迅速扩大的治疗选择。© 2023版权所有。由Elsevier有限公司出版。

Waldenström macroglobulinemia (WM) is a unique CD20+, B-cell non-Hodgkin lymphoma, characterized by lymphoplasmacytic infiltration of the bone marrow and circulating monoclonal immunoglobulin M. The clinical manifestations and outcomes of patients are highly variable. High-level evidence supports integration of monoclonal anti-CD20 antibody, rituximab, to the chemotherapy backbone to treat WM. However, its contemporary management has become more nuanced, with deeper understanding of the pathophysiology and incorporation of Bruton's tyrosine kinase (BTK) inhibitors to the treatment paradigm. Prior knowledge of the patients' MYD88L265P and CXCR4 mutation status may aid in the treatment decision-making. Currently, the two frequently utilized approaches include fixed-duration chemoimmunotherapy and BTK inhibitor-based continuous treatment until progression. Randomized trials comparing these two vastly divergent approaches are lacking. Recent studies demonstrating efficacy of B cell lymphoma-2 (BCL2) inhibitors and non-covalent BTK inhibitors in patients, previously exposed to a covalent BTK inhibitor, are a testament to the rapidly expanding options against WM.Copyright © 2023. Published by Elsevier Ltd.