Waldenstrom macroglobulinemia: 2015 update on diagnosis, risk stratification, and management
Waldenstrom macroglobulinemia: 2015 update on diagnosis, risk stratification, and management
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DOI：
10.1002/ajh.23922
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发表时间：
2015-04-01
影响因子：
12.8
通讯作者：
Gertz, Morie A.
中科院分区：
文献类型：
--
作者：
Gertz, Morie A.
疾病概述：瓦尔登斯特伦巨球蛋白血症（WM）是一种淋巴浆细胞淋巴瘤与免疫球蛋白M（IgM）单克隆蛋白。临床特征包括贫血、血小板减少、肝脾肿大、淋巴结病和罕见的高粘血症。诊断：骨髓中IgM单克隆蛋白与>= 10%的克隆性淋巴浆细胞相关可确诊。MYD 88的L265 P突变在超过90%的患者中可检测到。风险分层：年龄、血红蛋白水平、血小板计数、β 2微球蛋白和单克隆IgM浓度是预后所需的特征。风险适应性治疗：并非所有符合WM标准的患者都需要治疗;这些患者可以观察到症状出现。基于利妥昔单抗的治疗几乎用于所有美国WM患者，并且可以与烷化剂或嘌呤核苷类似物（或两者）联合使用。首选的马约诊所非研究治疗诱导是利妥昔单抗、环磷酰胺和地塞米松。未来的干细胞移植应考虑在诱导治疗的选择。难治性疾病的管理：硼替佐米，沙利度胺，依维莫司，依维莫替尼，卡非佐米，来那度胺，苯达莫司汀都已被证明在WM中具有活性。鉴于WM的自然病程，减少并发症将是未来治疗试验的优先事项。(C)2015 Wiley Periodicals，Inc.
Disease Overview: Waldenstrom macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy, and rarely hyperviscosity.Diagnosis: Presence of IgM monoclonal protein associated with >= 10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis. The L265P mutation in MYD88 is detectable in more than 90% of patients.Risk Stratification: Age, hemoglobin level, platelet count, beta(2) microglobulin, and monoclonal IgM concentrations are characteristics required for prognosis.Risk-Adapted Therapy: Not all patients who fulfill WM criteria require therapy; these patients can be observed until symptoms develop. Rituximab-based therapy is used in virtually all US patients with WM and can be combined with alkylating agent or purine nucleoside analog (or both). The preferred Mayo Clinic nonstudy therapeutic induction is rituximab, cyclophosphamide, and dexamethasone. Future stem cell transplantation should be considered in induction therapy selection.Management of Refractory Disease: Bortezomib, thalidomide, everolimus, ibrutinib, carfilzomib, lenalidomide, and bendamustine have all been shown to have activity in WM. Given WM's natural history, reduction of complications will be a priority for future treatment trials. (C) 2015 Wiley Periodicals, Inc.