Division of Hematology, Athens Medical Center, Athens, Greece.
Department of Clinical Therapeutics, National and Kapodistrian University of Athens, Athens, Greece.
Expert Rev Hematol. 2021 Jul;14(7):621-632. doi: 10.1080/17474086.2021.1947236. Epub 2021 Jul 2.
: Waldenström's macroglobulinemia (WM) is a rare lymphoma characterized by the accumulation of IgM-secreting lymphoplasmacytic cells in the bone marrow and other organs. Clinical sequelae relate to direct tissue infiltration by malignant cells but also to the physicochemical and immunological properties of the monoclonal IgM, resulting in a variety of disease-related complications.: This narrative review, following a thorough Pubmed search of pertinent published literature, discusses complications secondary to WM, related to direct tumor infiltration, monoclonal IgM circulation, and deposition, as well as other less common ones. The description and pathophysiology of these complications were described together with their specific management strategies and in the context of available treatment options for WM (anti-CD20 monoclonal antibody-based combinations, proteasome inhibitors, BTK inhibitors, and other emerging ones).: The availability of many novel, active and less toxic regimens for the treatment of WM allows the management of the disease with strategies that depend on clinical presentation and disease-related complications, age, toxicity considerations, and presence of comorbidities.
华氏巨球蛋白血症（WM）是一种罕见的淋巴瘤，其特征是骨髓和其他器官中积累了分泌 IgM 的淋巴浆细胞。临床后遗症不仅与恶性细胞的直接组织浸润有关，还与单克隆 IgM 的物理化学和免疫学特性有关，导致各种与疾病相关的并发症。
本综述文章通过全面的 Pubmed 检索相关已发表文献，讨论了与 WM 相关的并发症，这些并发症与直接肿瘤浸润、单克隆 IgM 循环和沉积以及其他较少见的并发症有关。本文描述了这些并发症的发病机制和病理生理学，并结合 WM 的可用治疗方案（基于抗 CD20 单克隆抗体的联合治疗、蛋白酶体抑制剂、BTK 抑制剂和其他新兴治疗方案）讨论了其具体的管理策略。
由于有许多新型、有效且毒性较低的 WM 治疗方案，因此可以根据临床表现和与疾病相关的并发症、年龄、毒性考虑因素以及合并症的存在，采用依赖于这些因素的策略来治疗 WM。
