Diagnosis and Management of Waldenstrom Macroglobulinemia: Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines
Diagnosis and Management of Waldenstrom Macroglobulinemia: Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines
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DOI：
10.4065/mcp.2010.0304
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发表时间：
2010-09-01
影响因子：
8.9
通讯作者：
Gertz, Morie A.
中科院分区：
文献类型：
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作者：
Ansell, Stephen M.;Kyle, Robert A.;Gertz, Morie A.
瓦尔登斯特伦巨球蛋白血症是一种B细胞恶性肿瘤，伴有骨髓或淋巴组织中的淋巴浆细胞浸润和血清中的单克隆免疫球蛋白M蛋白（IgM）。目前的治疗方法无法治愈，治疗患者的决定以及治疗方法的选择可能很复杂。使用风险适应的方法，我们提供治疗的时机和选择的建议。对于郁积型或无症状的Waldenstrom巨球蛋白血症和保留的血液学功能的患者，应观察而不进行治疗。患有中度血液学损害、需要治疗的IgM相关神经病变或对皮质类固醇无反应的溶血性贫血的症状性患者应单独接受标准剂量的利妥昔单抗，无需维持治疗。有严重全身症状、严重血液学损害、症状性巨大疾病或高粘血症的患者应接受DRC（地塞米松、利妥昔单抗、环磷酰胺）方案治疗。任何有高粘血症症状的病人首先应进行血浆置换治疗。对于对初始治疗有反应超过2年后复发的患者，应重复初始治疗。对于对初始治疗反应不足或反应持续时间少于2年的患者，应使用替代药物或联合用药。在所有符合条件的复发性疾病患者中应考虑自体干细胞移植。
Waldenstrom macroglobulinemia is a B-cell malignancy with lymphoplasmacytic infiltration in the bone marrow or lymphatic tissue and a monoclonal immunoglobulin M protein (IgM) in the serum. It is incurable with current therapy, and the decision to treat patients as well as the choice of treatment can be complex. Using a risk-adapted approach, we provide recommendations on timing and choice of therapy. Patients with smoldering or asymptomatic Waldenstrom macroglobulinemia and preserved hematologic function should be observed without therapy. Symptomatic patients with modest hematologic compromise, IgM-related neuropathy that requires therapy, or hemolytic anemia unresponsive to corticosteroids should receive standard doses of rituximab alone without maintenance therapy. Patients who have severe constitutional symptoms, profound hematologic compromise, symptomatic bulky disease, or hyperviscosity should be treated with the DRC (dexamethasone, rituximab, cyclophosphamide) regimen. Any patient with symptoms of hyperviscosity should first be treated with plasmapheresis. For patients who experience relapse after a response to initial therapy of more than 2 years' duration, the original therapy should be repeated. For patients who had an inadequate response to initial therapy or a response of less than 2 years' duration, an alternative agent or combination should be used. Autologous stem cell transplant should be considered in all eligible patients with relapsed disease.