利妥昔单抗治疗伴有IgM单克隆丙种球蛋白病的多发性神经病

Rituximab for polyneuropathy with IgM monoclonal gammopathy.

作者信息

Niermeijer J M F, Eurelings M, Lokhorst H L, van der Pol W-L, Franssen H, Wokke J H J, Notermans N C

机构信息

Department of Neurology and Rudolf Magnus Institute of Neurosciences, University Medical Center Utrecht, Utrecht, The Netherlands.

出版信息

J Neurol Neurosurg Psychiatry. 2009 Sep;80(9):1036-9. doi: 10.1136/jnnp.2008.155325.

DOI:10.1136/jnnp.2008.155325

PMID:19684235

Abstract

BACKGROUND

Polyneuropathy with IgM monoclonal gammopathy can be a disabling disorder necessitating treatment.

METHODS

In a prospective open label trial, 17 patients with disabling IgM MGUS polyneuropathy were treated with rituximab, a chimeric anti-CD-20 monoclonal antibody.

RESULTS

Rituximab induced an improvement of >or=1 point on the Overall Disability Sum Score in 2/17 patients, an improvement of >or=5% of the distal MRC sum score in 4/17 and the sensory sum score in 9/17 patients. Bone marrow investigations showed CD 20 B cell depletion in all patients. There were no serious adverse events. Compared with treatment with intermittent cyclophosphamide with prednisone or treatment with fludarabine, it shows a comparable response percentages but fewer side effects. The presence of anti-MAG and a disease duration shorter than 10 years may predict treatment response.

CONCLUSION

Rituximab is a candidate for treatment of IgM MGUS polyneuropathy and should be further investigated in a double-blind randomised trial.

摘要

背景

伴有IgM单克隆丙种球蛋白病的多发性神经病可能是一种需要治疗的致残性疾病。

方法

在一项前瞻性开放标签试验中，17例患有致残性IgM MGUS多发性神经病的患者接受了利妥昔单抗（一种嵌合抗CD - 20单克隆抗体）治疗。

结果

利妥昔单抗使2/17的患者总体残疾总分提高≥1分，4/17的患者远端MRC总分提高≥5%，9/17的患者感觉总分提高。骨髓检查显示所有患者的CD 20 B细胞均减少。未发生严重不良事件。与间歇环磷酰胺联合泼尼松治疗或氟达拉滨治疗相比，其反应率相当，但副作用更少。抗MAG的存在以及病程短于10年可能预测治疗反应。