研究动态
Articles below are published ahead of final publication in an issue. Please cite articles in the following format: authors, (year), title, journal, DOI.

华氏巨球蛋白血症 - 最先进的综述:第 1 部分:流行病学、发病机制、临床病理特征、鉴别诊断、风险分层和临床问题。

Waldenström Macroglobulinemia - A State-of-the-Art Review: Part 1: Epidemiology, Pathogenesis, Clinicopathologic Characteristics, Differential Diagnosis, Risk Stratification, and Clinical Problems.

发表日期:2024
作者: Michele Bibas, Shayna Sarosiek, Jorge J Castillo
来源: Bone & Joint Journal

摘要:

华氏巨球蛋白血症 (WM) 是淋巴瘤的一种罕见变异,被分类为 B 细胞恶性肿瘤,通过 IgM 副蛋白的存在、骨髓中克隆性小淋巴浆细胞 B 细胞浸润以及 MYD88 L265P 突变(在超过90%的案例。恶性细胞直接侵入淋巴结和脾脏等组织,加上与 IgM 相关的免疫反应,还可导致各种健康并发症,如血细胞减少、高粘血症、周围神经病变、淀粉样变性和 Bing-Neel 综合征。化学免疫疗法历来被认为是 WM 的首选治疗方法,其中利妥昔单抗与核苷类似物、烷化药物或蛋白酶体抑制剂的组合在抑制肿瘤生长方面表现出显着的功效。最近的研究提供的证据表明,布鲁顿酪氨酸激酶抑制剂(BTKI)无论是单独使用还是与其他药物联合使用,都已被证明在治疗 WM 方面是有效且安全的。该疾病被认为无法治愈,中位预期寿命为 10 至 12 年。
Waldenström macroglobulinemia (WM) is an infrequent variant of lymphoma, classified as a B-cell malignancy identified by the presence of IgM paraprotein, infiltration of clonal, small lymphoplasmacytic B cells in the bone marrow, and the MYD88 L265P mutation, which is observed in over 90% of cases. The direct invasion of the malignant cells into tissues like lymph nodes and spleen, along with the immune response related to IgM, can also lead to various health complications, such as cytopenias, hyperviscosity, peripheral neuropathy, amyloidosis, and Bing-Neel syndrome. Chemoimmunotherapy has historically been considered the preferred treatment for WM, wherein the combination of rituximab and nucleoside analogs, alkylating drugs, or proteasome inhibitors has exhibited notable efficacy in inhibiting tumor growth. Recent studies have provided evidence that Bruton Tyrosine Kinase inhibitors (BTKI), either used independently or in conjunction with other drugs, have been shown to be effective and safe in the treatment of WM. The disease is considered to be non-curable, with a median life expectancy of 10 to 12 years.