Clinical Hospital Dr Manuel Quintela, University of the Republic, British Hospital, Montevideo, Uruguay.
Irmandade Da Santa Casa De Misericordia De São Paulo, Sao Paulo, Brazil.
Hematol Oncol Clin North Am. 2023 Aug;37(4):801-807. doi: 10.1016/j.hoc.2023.04.010. Epub 2023 May 29.
Waldenström macroglobulinemia (WM) is a rare, indolent, and currently incurable B-cell neoplasm characterized by monoclonal immunoglobulin M gammopathy, frequent nodal involvement, and lymphoplasmacytic infiltration of the bone marrow. The clinical pattern at diagnosis is similar to that reported in developed countries but, unfortunately, the tools for a complete diagnosis and access to novel therapies are suboptimal. Older drugs such as bendamustine, cyclophosphamide, and chlorambucil may still play a role in treating WM. Prospective studies in resource-limited regions are required to further evaluate these essential aspects of the disease. In this document, we issue recommendations based on our local reality.
华氏巨球蛋白血症（WM）是一种罕见的、惰性的、目前无法治愈的 B 细胞肿瘤，其特征是单克隆免疫球蛋白 M 血症、频繁的淋巴结受累以及骨髓中的淋巴浆细胞浸润。诊断时的临床模式与发达国家报道的相似，但不幸的是，用于全面诊断和获得新型疗法的工具并不理想。苯达莫司汀、环磷酰胺和苯丁酸氮芥等较老的药物在治疗 WM 方面可能仍发挥作用。需要在资源有限的地区进行前瞻性研究，以进一步评估该疾病的这些重要方面。在本文件中，我们根据当地实际情况提出建议。
