Vela-Ojeda J, García-Ruiz Esparza M A, Padilla-González Y, Rosas-Cabral A, García-Chávez J, Xolotl-Castillo M, Salazar-Exaire D, Arenas-Osuna J, Aviña-Zubieta J A, Vadillo-Buenfil M, Abraham-Majluf S
Department of Hematology, Hospital de Especialidades Centro Médico La Raza, Instituto Mexicano del Seguro Social, México DF, México.
J Interferon Cytokine Res. 2002 Oct;22(10):1013-6. doi: 10.1089/107999002760624233.
Waldenström's macroglobulinemia is a rare malignant disorder of B lymphocytes. There are no studies on the use of interferon-alpha (IFN-alpha) as frontline therapy in this disease. Between April 1991 and September 2000, we treated 21 newly diagnosed patients using 8 mg/m(2) chlorambucil and 40 mg/m(2) prednisone p.o. daily for 10 days and 3 megaU/m(2) IFN-alpha three times a week. Patients who responded after induction continued receiving IFN until relapse or death. We found a high frequency of peripheral neuropathy (43%) and grade 3 diffuse marrow fibrosis (43%). Objective response was achieved in 12 (57%) patients, including 4 (19%) complete responders. Median time from treatment to response was 8 months (range 3-18). Median progression-free survival was 70 months (95% CI 47-93), and overall survival was 91 months (95% CI 50-132). Patients who achieved objective response lived longer (91 vs. 33 months, p < 0.03), as did patients who had lactic dehydrogenase (LDH) < 180 U/L (89 vs. 54 months, p < 0.01). Grade 3 hematologic toxicity was observed during induction in 5 patients. IFN-alpha is an effective agent for the induction and maintenance treatment of Waldenström's macroglobulinemia patients. LDH > 180 U/L and failure to respond are adverse prognostic factors.
华氏巨球蛋白血症是一种罕见的B淋巴细胞恶性疾病。目前尚无关于将α干扰素（IFN-α）作为该疾病一线治疗方法的研究。1991年4月至2000年9月期间，我们对21例新诊断患者采用每日口服8mg/m²苯丁酸氮芥和40mg/m²泼尼松，持续10天，并每周三次皮下注射3百万单位/m²IFN-α进行治疗。诱导治疗后有反应的患者继续接受IFN治疗，直至复发或死亡。我们发现外周神经病变（43%）和3级弥漫性骨髓纤维化（43%）的发生率很高。12例（57%）患者获得客观缓解，其中4例（19%）完全缓解。从治疗到缓解的中位时间为8个月（范围3 - 18个月）。中位无进展生存期为70个月（95%CI 47 - 93），总生存期为91个月（95%CI 50 - 132）。获得客观缓解的患者生存期更长（91个月对33个月，p < 0.03），乳酸脱氢酶（LDH）< 180 U/L的患者也是如此（89个月对54个月，p < 0.01）。5例患者在诱导治疗期间出现3级血液学毒性。IFN-α是诱导和维持治疗华氏巨球蛋白血症患者的有效药物。LDH > 180 U/L和无反应是不良预后因素。
