Rotoli B, De Renzo A, Frigeri F, Buffardi S, Marcenò R, Cavallaro A M, Ruggeri P, Liso V, Musto P, Andriani A
Leuk Lymphoma. 1994 May;13(5-6):463-9. doi: 10.3109/10428199409049636.
Waldenström's macroglobulinemia (WM) is an incurable disorder of B cells. Following occasional reports of response to alpha interferon (IFN) and in view of its effectiveness in hairy cell leukemia, we tested this agent in a relatively large group (n = 88) of patients who had an IgM monoclonal component (MC) greater than 10 g/l. Thirty eight patients had a MC > 30 g/l and were classified as Waldenström's macroglobulinemia (WM), while fifty had either WM in an early stage or an IgM monoclonal gammopathy of undeterminated significance (all of them operationally classified as IgM-MGUS). All patients received IFN 3 MU/day for one month and then 3 times/week. Response to treatment was mainly based on MC reduction in two consecutive determinations (> 50%: major response; 25-50%: minor response). Of 36 evaluable WM patients, 12 had a major and 6 a minor response; of 41 evaluable IgM-MGUS patients, 2 had a major and 6 a minor response. In WM patients with a major response, MC reduction was associated with disappearance of hyperviscosity symptoms, raised Hb level and reduced bone marrow lymphoplasmacytosis. At the dose used, tolerance was excellent in the majority of patients; only 15% withdrew from the study due to side effects. Although single cases and very small series have already been reported, no large study collecting quantitative data on the effects of alpha IFN in WM has been published so far. Our results suggest that IFN treatment is not indicated for patients with a low monoclonal component, while it is of clinical benefit in about 50% of patients with IgM > 30 g/l.
华氏巨球蛋白血症（WM）是一种无法治愈的B细胞疾病。在偶尔有关于对α干扰素（IFN）有反应的报道后，鉴于其对毛细胞白血病的有效性，我们在一组相对较大的（n = 88）IgM单克隆成分（MC）大于10 g/l的患者中测试了这种药物。38例患者的MC> 30 g/l，被归类为华氏巨球蛋白血症（WM），而50例患者处于WM早期或意义未明的IgM单克隆丙种球蛋白病（所有患者在操作上均归类为IgM-MGUS）。所有患者每天接受3 MU的IFN治疗一个月，然后每周3次。治疗反应主要基于连续两次测定中MC的降低（> 50%：主要反应；25 - 50%：次要反应）。在36例可评估的WM患者中，12例有主要反应，6例有次要反应；在41例可评估的IgM-MGUS患者中，2例有主要反应，6例有次要反应。在有主要反应的WM患者中，MC降低与高黏滞症状消失、血红蛋白水平升高和骨髓淋巴浆细胞增多减少相关。在所使用的剂量下，大多数患者耐受性良好；只有15%的患者因副作用退出研究。尽管已经报道了个别病例和非常小的系列研究，但迄今为止尚未发表收集关于α干扰素对WM影响的定量数据的大型研究。我们的结果表明，IFN治疗不适用于单克隆成分低的患者，而对于约50%的IgM> 30 g/l的患者具有临床益处。
