Dimopoulos Meletios A, Anagnostopoulos Athanasios
Department of Clinical Therapeutics, University of Athens School of Medicine, Alexandra Hospital, 80 Vas. Sofias, Athens, 11528, Greece.
Curr Treat Options Oncol. 2007 Apr;8(2):144-53. doi: 10.1007/s11864-007-0016-2.
Waldenstrom's macroglobulinemia is defined by bone marrow lymphoplasmacytic infiltration and by production of monoclonal IgM. Treatment is employed only to symptomatic patients. Alkylating agents (chlorambucil), nucleoside analogues and rituximab are reasonable choices for primary therapy. Combination therapy either with nucleoside analogues with alkylating agents and/or rituximab or rituximab with chemotherapy such as CHOP or cyclophosphamide are also reasonable frontline treatment options for WM patients. Several factors should be taken into account when choosing the most appropriate primary treatment. These factors include the age of the patient and possible co-morbidities, the presence of cytopenias and especially thrombocytopenia, the presence of symptoms and signs indicative of hyperviscosity, the need for rapid disease control due to severe symptoms, significant splenomegaly or lymphadenopathy, symptomatic peripheral neuropathy and whether the patient is candidate for autologous stem cell transplantation. For patients with refractory or relapsing disease, the use of an alternate first-line agent is reasonable. Outside the setting of a clinical trial, the administration of high-dose therapy should be reserved only for patients refractory to alkylating agents, purine nucleoside and rituximab. For patients who develop resistance to all three classes of agents, alemtuzumab, thalidomide with or without dexamethasone or bortezomib could be tried.
华氏巨球蛋白血症的定义为骨髓淋巴细胞浆细胞浸润以及单克隆IgM的产生。仅对有症状的患者进行治疗。烷化剂（苯丁酸氮芥）、核苷类似物和利妥昔单抗是一线治疗的合理选择。核苷类似物与烷化剂和/或利妥昔单抗联合治疗，或利妥昔单抗与化疗（如CHOP或环磷酰胺）联合治疗，也是WM患者合理的一线治疗选择。选择最合适的一线治疗时应考虑几个因素。这些因素包括患者的年龄和可能的合并症、血细胞减少尤其是血小板减少的存在、高黏滞血症症状和体征的存在、因严重症状、显著脾肿大或淋巴结病、有症状的周围神经病变而需要快速控制疾病，以及患者是否适合自体干细胞移植。对于难治性或复发性疾病患者，使用替代的一线药物是合理的。在临床试验之外，高剂量治疗仅应保留给对烷化剂、嘌呤核苷和利妥昔单抗难治的患者。对于对所有三类药物产生耐药性的患者，可以尝试使用阿仑单抗、沙利度胺（加或不加地塞米松）或硼替佐米。
