Sekiguchi Naohiro
Department of Hematology, National Hospital Organization Disaster Medical Center.
Rinsho Ketsueki. 2021;62(8):1139-1148. doi: 10.11406/rinketsu.62.1139.
Waldenström's macroglobulinemia or lymphoplasmacytic lymphoma (WM/LPL) is a rare subtype of indolent B-cell lymphoma with plasmacytic differentiation. Owing to its rarity, the pathogenesis, biology, and standard of care have not been established. In 2012 the MYD88 L265P mutation is proven as the major oncogenesis in WM/LPL; therefore, the pathogenesis and underlying biology of WM/LPL have been drastically explored. Furthermore, treatment options have also been developed, and Bruton's tyrosine kinase (BTK) inhibitor has been recently approved for untreated and relapsed/refractory WM/LPL in August 2020 in Japan. In this article, after a brief review of the clinical and biological characteristics of WM/LPL, we discuss the ideal therapeutic algorithm, including novel BTK inhibitor.
华氏巨球蛋白血症或淋巴浆细胞性淋巴瘤（WM/LPL）是一种罕见的惰性B细胞淋巴瘤亚型，具有浆细胞分化。由于其罕见性，其发病机制、生物学特性及治疗标准尚未确立。2012年，MYD88 L265P突变被证实是WM/LPL的主要致癌因素；因此，对WM/LPL的发病机制及潜在生物学特性进行了深入研究。此外，治疗方案也有所发展，布鲁顿酪氨酸激酶（BTK）抑制剂于2020年8月在日本被批准用于未经治疗及复发/难治性WM/LPL。在本文中，在简要回顾WM/LPL的临床和生物学特征后，我们讨论了理想的治疗方案，包括新型BTK抑制剂。
