Sekiguchi Naohiro
Division of Hematology, National Hospital Organization Disaster Medical Center.
Rinsho Ketsueki. 2019;60(8):988-997. doi: 10.11406/rinketsu.60.988.
Waldenström macroglobulinemia (WM) is a rare low-grade B-cell lymphoma. This report focuses on WM in Japan. Regarding epidemiology, per the 2016 registry data by the Japanese Society of Hematology, WM and lymphoplasmacytic lymphoma (LPL) cases were 229 and 125, respectively (1.97% of 17,957 mature lymphoid malignancies), with the annual incidence of WM/LPL in Japan 2.8 per million. In addition, 6q deletion (6q del) is the leading aberration and one of the poor prognostic factors in WM. Our findings suggested that the serum IgM level was higher in WM with 6q del compared with those without, and the B-cell receptor signaling pathway and IL-21 receptor expression were activated in WM with 6q del. Hence, these findings might be attributed to the aggressiveness of the WM with 6q del. Regarding treatment, recent studies investigating the epidemiological treatment pattern for WM have reported rituximab (R) monotherapy and alkylating regimen (±R) as the leading initial treatment. Nevertheless, ideal treatment algorithm in Japan warrants further investigation.
华氏巨球蛋白血症（WM）是一种罕见的低度B细胞淋巴瘤。本报告聚焦于日本的WM。关于流行病学，根据日本血液学会2016年的登记数据，WM和淋巴浆细胞淋巴瘤（LPL）病例分别为229例和125例（占17957例成熟淋巴oid恶性肿瘤的1.97%），日本WM/LPL的年发病率为百万分之2.8。此外，6q缺失（6q del）是WM的主要畸变及不良预后因素之一。我们的研究结果表明，与无6q del的WM相比，有6q del的WM患者血清IgM水平更高，且有6q del的WM中B细胞受体信号通路及IL-21受体表达被激活。因此，这些发现可能归因于有6q del的WM具有侵袭性。关于治疗，近期调查WM流行病学治疗模式的研究报告称利妥昔单抗（R）单药治疗及烷化剂方案（±R）是主要的初始治疗方法。然而，日本理想的治疗方案仍有待进一步研究探索。
