[Therapeutic algorithm of Waldenström's macroglobulinemia or lymphoplasmacytic lymphoma in Japan]

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.