PDF(Pubmed)
Abstract：
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.
摘要：
Waldenström巨球蛋白血症（WM）是淋巴瘤的罕见变种，通过IgM副蛋白的存在鉴定为B细胞恶性肿瘤，克隆的浸润，骨髓中的小淋巴浆细胞B细胞，和MYD88L265P突变，在超过90％的病例中观察到。恶性细胞直接侵入淋巴结和脾脏等组织，伴随着与IgM相关的免疫反应，还会导致各种健康并发症，比如血细胞减少症，高粘度,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。其中利妥昔单抗和核苷类似物的组合，烷化药物，或蛋白酶体抑制剂在抑制肿瘤生长方面表现出显著的功效。最近的研究提供了证据，布鲁顿酪氨酸激酶抑制剂（BTKI），独立使用或与其他药物联合使用，已被证明在治疗WM中是有效和安全的。这种疾病被认为是不可治愈的，平均预期寿命为10至12岁。
