Leblond Véronique, Choquet Sylvain
Hôpital Pitié Salpêtriére, Paris, France.
Semin Oncol. 2003 Apr;30(2):239-42. doi: 10.1053/sonc.2003.50040.
Waldenstrom's macroglobulinemia (WM), a rare B-cell malignancy, is incurable. Conventional treatment consists of alkylating agents (especially chlorambucil), with or without corticosteroids. Purine analogues such as fludarabine are also active. Response rates to first-line therapy range from 38% to 85%. Discrepancies in response rates between different studies could be due to the small patient populations in two studies and to differences in patient characteristics and response criteria. Since 1990, several phase 2 trials of purine analogues have been done with previously treated patients; fludarabine induced responses in about one third of patients who were resistant to previous treatments. Response rates to fludarabine in previously treated patients range from 30% to 50% and are highest among patients who are still sensitive to their primary therapy. The responses last from 32 to 41 months. The principal toxicity of fludarabine is myelosuppression. Trials of fludarabine combination therapy with drugs such as rituximab are ongoing.
华氏巨球蛋白血症(WM)是一种罕见的B细胞恶性肿瘤,无法治愈。传统治疗方法包括使用烷化剂(尤其是苯丁酸氮芥),可联合或不联合使用皮质类固醇。嘌呤类似物如氟达拉滨也有活性。一线治疗的缓解率在38%至85%之间。不同研究之间缓解率的差异可能是由于两项研究中的患者群体较小,以及患者特征和缓解标准的差异。自1990年以来,已经对先前接受过治疗的患者进行了几项嘌呤类似物的2期试验;氟达拉滨在约三分之一对先前治疗耐药的患者中诱导出缓解。先前接受过治疗的患者对氟达拉滨的缓解率在30%至50%之间,在对初始治疗仍敏感的患者中最高。缓解持续32至41个月。氟达拉滨的主要毒性是骨髓抑制。氟达拉滨与利妥昔单抗等药物联合治疗的试验正在进行中。