文章：

Waldenström巨球蛋白血症治疗算法2018

Waldenström macroglobulinemia treatment algorithm 2018

原文发布日期： 2018-05-01

DOI: 10.1038/s41408-018-0076-5

类型: Current Treatment Algorithm

开放获取: 是

英文摘要：

Waldenström macroglobulinemia is often an indolent disorder, and many patients are candidates for observation with careful monitoring. For symptomatic patients, one must distinguish between those patients whose symptoms are related to immunologic manifestations associated with the IgM monoclonal protein and those that have symptoms related to progressive marrow and nodal infiltration with lymphoplasmacytic lymphoma. In Waldenström macroglobulinemia, the driver for therapy in the majority of patients is progressive anemia, secondary to bone marrow replacement by lymphoplasmacytic lymphoma. Recent introduction of MYD88 mutational analysis has been very useful for diagnostic purposes but is unclear what effect it might have on the prognosis or response rate to therapy. An algorithm is provided on the management of asymptomatic individuals and the sequence used for chemotherapeutic intervention of symptomatic patients.

摘要翻译： 

华氏巨球蛋白血症通常是一种惰性病程，许多患者适合采取密切监测下的观察策略。对于有症状的患者，必须区分其症状是与IgM单克隆蛋白相关的免疫学表现有关，还是与淋巴浆细胞淋巴瘤导致的进行性骨髓及淋巴结浸润有关。在该疾病中，大多数患者的治疗驱动因素是进行性贫血，这是淋巴浆细胞淋巴瘤取代骨髓所引起的继发性病变。近期应用的MYD88基因突变分析对诊断具有重要价值，但其对预后及治疗缓解率的影响尚不明确。本文提供了无症状患者的诊疗流程，以及有症状患者化疗干预的序贯方案。

原文链接：

Waldenström macroglobulinemia treatment algorithm 2018