Division of Hematology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.
Am J Hematol. 2012 May;87(5):503-10. doi: 10.1002/ajh.23192.
Waldenström macroglobulinemia (WM) is a lymphoplasmacytic lymphoma with immunoglobulin M (IgM) monoclonal protein. Clinical features include anemia, thrombocytopenia, hepatosplenomegaly, and lymphadenopathy.
Presence of IgM monoclonal protein associated with ≥10% clonal lymphoplasmacytic cells in bone marrow confirms the diagnosis.
Age, hemoglobin level, platelet count, β(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.
Bortezomib, thalidomide, 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.
华氏巨球蛋白血症（WM）是一种淋巴浆细胞淋巴瘤，伴有免疫球蛋白 M（IgM）单克隆蛋白。临床特征包括贫血、血小板减少、肝脾肿大和淋巴结病。
骨髓中存在 IgM 单克隆蛋白，伴有≥10%的克隆性淋巴浆细胞即可确诊。
年龄、血红蛋白水平、血小板计数、β(2)微球蛋白和单克隆 IgM 浓度是用于预后的特征。
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，直到出现症状。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。梅奥诊所非研究性的首选治疗诱导方案是利妥昔单抗、环磷酰胺和地塞米松。未来应在诱导治疗选择中考虑干细胞移植。
硼替佐米、沙利度胺、来那度胺和苯达莫司汀均已显示在 WM 中有活性。鉴于 WM 的自然病史，减少并发症将是未来治疗试验的优先事项。
