Department of Internal Medicine, UMC Utrecht, Utrecht, The Netherlands.
Université Paris Cité, Assistance Publique & Hôpitaux de Paris, Paris, France.
Hematol Oncol. 2024 Nov;42(6):e3210. doi: 10.1002/hon.3210. Epub 2023 Jul 17.
Marginal Zone Lymphoma (MZL) and Waldenström's Macroglobulinemia (WM) are indolent lymphomas that both arise from post germinal center lymphocytes. Both can secrete a monoclonal protein but high levels are mostly only seen in WM. The MYD88 L256P somatic mutation that is present in an estimated 95% of patients with WM has helped greatly in differentiating the two lymphomas. Several large clinical studies with new drugs have been performed that have provided new treatment options for both MZL and WM patients. In this short review we will discuss the recent literature published and provide some recommendations.
边缘区淋巴瘤(MZL)和华氏巨球蛋白血症(WM)是惰性淋巴瘤,均起源于生发中心后淋巴细胞。两者均可分泌单克隆蛋白,但高水平主要仅见于 WM。MYD88 L256P 体细胞突变存在于估计 95%的 WM 患者中,极大地有助于区分这两种淋巴瘤。已经进行了几项大型临床试验,这些试验为 MZL 和 WM 患者提供了新的治疗选择。在这篇简短的综述中,我们将讨论最近发表的文献并提供一些建议。