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意义未明的IgG λ单克隆丙种球蛋白病(MGUS)相关慢性复发性多神经根神经病——卡莫司汀治疗后完全缓解

Chronic relapsing polyradiculoneuropathy in IgG lambda monoclonal gammopathy of undetermined significance (MGUS)--complete remission following carmustine treatment.

作者信息

Wöber C, Schmidbauer M, Podreka I, Wenger S, Pafflmeyer N, Deecke L

机构信息

Universitätsklinik für Neurologie, Wien.

出版信息

Wien Klin Wochenschr. 1995;107(10):318-20.

PMID:7785279
Abstract

A previously healthy 43 year-old female developed IgG lambda monoclonal gammopathy of undetermined significance (MGUS) and ascending sensorimotor polyradiculoneuropathy which relapsed 11 times within 2 years. Marked improvement was noted repeatedly after plasmapheresis. However, on each occasion symptoms and signs of polyradiculoneuropathy recurred almost exactly 3 weeks after plasma-pheresis. Following 6 weeks of treatment with carmustine (70 mg/week), nearly complete recovery was established, which has persisted up to now (82 months after the end of therapy). The close temporal correlation between clinical relapse and recurrence of the IgG paraprotein and its permanent absence in stable clinical remission after carmustine treatment suggest a causal relationship between the paraprotein and the polyradiculoneuropathy. However, further studies are required to confirm this observation, as well as the efficacy of carmustine therapy.

摘要

一名既往健康的43岁女性患意义未明的IgG λ单克隆丙种球蛋白病(MGUS)及感觉运动性上升性多神经根神经病,该病在2年内复发11次。血浆置换后多次观察到明显改善。然而,每次血浆置换后几乎正好3周,多神经根神经病的症状和体征就会复发。在用卡莫司汀(70mg/周)治疗6周后,病情几乎完全恢复,且一直持续至今(治疗结束后82个月)。临床复发与IgG副蛋白复发之间密切的时间相关性以及卡莫司汀治疗后临床稳定缓解期副蛋白的永久消失提示副蛋白与多神经根神经病之间存在因果关系。然而,需要进一步研究来证实这一观察结果以及卡莫司汀治疗的疗效。

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