文章：

Waldenstrom巨球蛋白血症：预后和管理

Waldenstrom macroglobulinemia: prognosis and management

原文发布日期：2015-03-27

DOI: 10.1038/bcj.2015.28

类型: Review

开放获取: 是

英文摘要：

Waldenstrom macroglobulinemia (WM) is a B-cell lymphoplasmacytic lymphoma characterized by monoclonal immunoglobulin M protein in the serum and infiltration of bone marrow with lymphoplasmacytic cells. Asymptomatic patients can be observed without therapy. First-line therapy should consist of the monoclonal anti-CD20 antibody, rituximab, given typically in combination with other agents. We prefer dexamethasone, rituximab, cyclophosphamide (DRC) as initial therapy for most patients with symptomatic WM. Other reasonable options are bortezomib, rituximab, dexamethasone (BoRD) or bendamustine plus rituximab (BR). All of these regimens are associated with excellent response and tolerability. Initial therapy is usually administered for 6 months, followed by observation. Response to therapy is assessed using the standard response criteria developed by the International Working Group on Waldenstrom macroglobulinemia. Relapse is almost inevitable in WM but may occur years after initial therapy. In symptomatic patients relapsing more than 1–2 years after initial therapy, the original treatment can be repeated. For relapse occurring sooner, an alternative regimen is used. In select patients, high-dose chemotherapy followed by autologous hematopoietic cell transplantation may be an option at relapse. Options for therapy of relapsed WM besides regimens used in the front-line setting include ibrutinib, purine nucleoside analogs (cladribine, fludarabine), carfilzomib and immunomodulatory agents (thalidomide, lenalidomide).

摘要翻译： 

华氏巨球蛋白血症（WM）是一种B细胞淋巴浆细胞性淋巴瘤，其特征为血清中出现单克隆免疫球蛋白M蛋白，以及骨髓中淋巴浆细胞浸润。无症状患者可无需治疗，仅进行观察。一线治疗应包含单克隆抗CD20抗体利妥昔单抗，通常与其他药物联合使用。对于大多数有症状的WM患者，我们首选地塞米松、利妥昔单抗、环磷酰胺（DRC）作为初始治疗方案。其他合理选择包括硼替佐米、利妥昔单抗、地塞米松（BoRD）或苯达莫司汀联合利妥昔单抗（BR）。所有这些方案均具有优异的疗效和耐受性。初始治疗通常持续6个月，随后进入观察期。治疗反应根据华氏巨球蛋白血症国际工作组制定的标准反应评估标准进行评估。WM患者几乎不可避免地会复发，但可能发生在初始治疗数年之后。对于初始治疗1-2年后复发的有症状患者，可重复原治疗方案。若复发时间更早，则需采用替代方案。对于部分精选患者，复发时可采用大剂量化疗联合自体造血细胞移植。除一线治疗方案外，复发WM的治疗选择还包括伊布替尼、嘌呤核苷类似物（克拉屈滨、氟达拉滨）、卡非佐米以及免疫调节剂（沙利度胺、来那度胺）。

原文链接：

Waldenstrom macroglobulinemia: prognosis and management