PLASMA-EXCHANGE IN POLYNEUROPATHY ASSOCIATED WITH MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE

PLASMA-EXCHANGE IN POLYNEUROPATHY ASSOCIATED WITH MONOCLONAL GAMMOPATHY OF UNDETERMINED SIGNIFICANCE
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DOI:
10.1056/nejm199111213252105
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发表时间:
1991-11-21
影响因子:
158.5
通讯作者:
KYLE, RA
KYLE, RA
中科院分区:
医学1区
文献类型:
--
作者:
DYCK, PJ;LOW, PA;KYLE, RA

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背景。与意义未明的单克隆丙种球蛋白病(MGUS)相关的多发性神经病已通过血浆置换、静脉注射免疫球蛋白和化疗进行治疗,但这些治疗的有效性仍不确定。方法。在一项双盲试验中,我们随机分配 39 名患有稳定或恶化的神经病以及 IgG、IgA 或 IgM 型 MGUS 的患者接受每周两次血浆置换,持续三周或接受假血浆置换。最初接受假血浆置换的患者随后在开放试验中接受血浆置换。结果。在双盲试验中,假交换组的平均神经病残疾评分较基线(从 62.5 至 60.5)提高了 2 分,血浆交换组则较基线提高了 12 分(从 58.3 至 46.3)(P = 0.06)。在肌无力评分(神经病残疾评分的一个组成部分)中也观察到类似的差异(分别改善 1 分和 10 分;P = 0.07)。治疗后,假交换组运动神经复合肌肉动作电位总和比基线低(差)1.2 mV,血浆交换组高 0.4 mV(好)(P = 0.07)。血浆置换带来的更大程度的改善等于或大于不能用脚跟或脚趾行走与能够进行这些活动之间的差异。振动检测阈值、运动神经传导速度和感觉神经动作电位的变化在治疗组之间没有显着差异。在开放试验中,最初接受假交换的患者接受血浆交换治疗,神经病残疾评分(P = 0.04)、无力评分(P = 0.07)和复合肌肉动作电位总和(P = 0.07)在血浆交换中比假交换中改善更多。在双盲和开放试验中,患有 IgG 或 IgA 丙种球蛋白病的患者对血浆置换的反应比患有 IgM 丙种球蛋白病的患者更好。结论。血浆置换似乎对 MGUS 相关的神经病有效,尤其是 IgG 或 IgA 型神经病。
Background. Polyneuropathy associated with monoclonal gammopathy of undetermined significance (MGUS) has been treated with plasma exchange, intravenous immune globulin, and chemotherapy, but the effectiveness of these treatments remains uncertain.Methods. We randomly assigned 39 patients with stable or worsening neuropathy and MGUS of the IgG, IgA, or IgM type to receive either plasma exchange twice weekly for three weeks or sham plasma exchange, in a double-blind trial. The patients who initially underwent sham plasma exchange subsequently underwent plasma exchange in an open trial.Results. In the double-blind trial, the average neuropathy disability score improved by 2 points from base line (from 62.5 to 60.5) in the sham-exchange group and by 12 points (from 58.3 to 46.3) in the plasma-exchange group (P = 0.06). A similar difference was observed in the weakness score, a component of the neuropathy disability score (improvement, 1 and 10 points, respectively; P = 0.07). After treatment the summed compound muscle action potentials of motor nerves were 1.2 mV lower (worse) than at base line in the sham-exchange group and 0.4 mV higher (better) in the plasma-exchange group (P = 0.07). The greater degree of improvement with plasma exchange was equal in magnitude to or greater than the difference between not being able to walk on the heels or toes and being able to perform these activities. Changes in the vibratory detection threshold, summed motor-nerve conduction velocity, and sensory-nerve action potentials did not differ significantly between the treatment groups. In the open trial, in which patients who initially underwent sham exchange were treated with plasma exchange, the neuropathy disability score (P = 0.04), weakness score (P = 0.07), and summed compound muscle action potentials (P = 0.07) improved more with plasma exchange than they had with sham exchange. In both the double-blind and the open trial, those with IgG or IgA gammopathy had a better response to plasma exchange than those with IgM gammopathy. Conclusions. Plasma exchange appears to be efficacious in neuropathy associated with MGUS, especially of he IgG or IgA type.