Gertz Morie A, Anagnostopoulos Athanasios, Anderson Kenneth, Branagan Andrew R, Coleman Morton, Frankel Stanley R, Giralt Sergio, Levine Todd, Munshi Nikhil, Pestronk Alan, Rajkumar Vincent, Treon Steven P
Mayo Clinic, Rochester, MN, USA.
Semin Oncol. 2003 Apr;30(2):121-6. doi: 10.1053/sonc.2003.50039.
This presentation represents consensus recommendations for the treatment of patients with Waldenstrom's macroglobulinemia (WM), which were prepared in conjunction with the second International Workshop held in Athens, Greece during September 2002. The faculty adopted the following statements for the management of patients with Waldenstrom's macroglobulinemia: (1) Alkylating agents, nucleoside analogues, and rituximab are reasonable choices for first line therapy of WM. (2) Both cladribine and fludarabine are reasonable choices for the therapy of WM. (3) Combinations of alkylating agents, nucleoside analogues, or rituximab should at this time be encouraged in the context of a clinical trial. (4) In WM, rituximab can cause a sudden rise in serum IgM and viscosity levels in certain patients, which may lead to complications, therefore close monitoring of these parameters and symptoms of hyperviscosity is recommended for WM patients undergoing rituximab therapy. (5) For relapsed disease, it is reasonable to use an alternate first line agent or re-use of the same agent; however, since autologous stem cell transplantation may have a role in treating patients with relapsed disease it is recommended that for patients in whom autologous transplantation is seriously being considered, exposure to alkylator or nucleoside analogue drugs should be limited. (6) Combination chemotherapy for patients who can tolerate myelotoxic therapy, thalidomide alone or with dexamethasone, can reasonably be considered to have relapsed. (7) Autologous stem cell transplantation may be considered for patients with refractory or relapsing disease. (8) Allogeneic transplantation should only be undertaken in the context of a clinical trial. (9) Plasmapheresis should be considered as interim therapy until definitive therapy can be initiated. (10) Rituximab should be considered for patients with IgM-related neuropathies. (11) Corticosteroids may be useful in the treatment of symptomatic mixed cryoglobulinemia. (12) Splenectomy is rarely indicated but has been used to manage painful splenomegaly and hypersplenism.
本报告代表了关于华氏巨球蛋白血症（WM）患者治疗的共识性建议，这些建议是在2002年9月于希腊雅典举行的第二届国际研讨会上共同制定的。与会人员就华氏巨球蛋白血症患者的管理采纳了以下声明：（1）烷化剂、核苷类似物和利妥昔单抗是WM一线治疗的合理选择。（2）克拉屈滨和氟达拉滨都是WM治疗的合理选择。（3）目前应鼓励在临床试验背景下联合使用烷化剂、核苷类似物或利妥昔单抗。（4）在WM中，利妥昔单抗可导致某些患者血清IgM和黏度水平突然升高，这可能会引发并发症，因此建议对接受利妥昔单抗治疗的WM患者密切监测这些参数和高黏滞症状。（5）对于复发疾病，使用替代一线药物或重新使用同一药物是合理的；然而，由于自体干细胞移植可能在治疗复发疾病患者中发挥作用，建议对于认真考虑自体移植的患者，应限制其接触烷化剂或核苷类似物药物。（6）对于能够耐受骨髓毒性治疗的复发患者，可合理考虑联合化疗、单独使用沙利度胺或与地塞米松联合使用。（7）对于难治性或复发性疾病患者，可考虑自体干细胞移植。（8）异基因移植仅应在临床试验背景下进行。（9）在开始确定性治疗之前，应考虑将血浆置换作为临时治疗。（10）对于患有IgM相关神经病变的患者，应考虑使用利妥昔单抗。（11）皮质类固醇可能有助于治疗有症状的混合性冷球蛋白血症。（12）脾切除术很少有指征，但已用于处理疼痛性脾肿大和脾功能亢进。
