Anti-myelin-associated glycoprotein antibody positive IgM monoclonal gammopathy related peripheral neuropathy: 11 cases and literature review
10.3760/cma.j.issn.0253-2727.2019.11.003
- VernacularTitle: 抗髓鞘相关糖蛋白抗体阳性IgM相关性周围神经病11例临床分析
- Author:
Xuemin GAO
1
;
Mingnan JIA
1
;
Min QIAN
2
;
Haitao REN
2
;
Lu ZHANG
1
;
Kaini SHEN
1
;
Xinxin CAO
1
;
Jian LI
1
Author Information
1. Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences, Beijing 100730, China
2. Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medicine Sciences, Beijing 100730, China
- Publication Type:Journal Article
- Keywords:
Anti-myelin-associated glycoprotein antibody;
Peripheral neuropathy;
Monoclonal gammopathy;
Waldenström macroglobulinaemia
- From:
Chinese Journal of Hematology
2019;40(11):901-905
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To improve the understanding of rare anti-myelin-associated glycoprotein (MAG) positive IgM monoclonal gammopathy related peripheral neuropathy (IgM-PN) .
Methods:Eleven cases of IgM paraproteinemia and anti-MAG antibody positive neuropathy diagnosed since 2014 in Peking Medical Union College Hospital were summarized. The medical records including clinical manifestation, lab results, treatment and prognosis were analyzed.
Results:Among the 11 patients (8 male and 3 female) , the median onset age is 63 years old (range from 52 to 77 years old) . The peripheral neuropathy of 9 patients were characterized by distal onset of numbness, 6 patients suffered from muscle weakness. The nerve conduction velocity study indicated that all 11 patients had demyelinating peripheral nerve damage, which was sensory predominant and more severe in lower limbs, 6 of them had secondary axonal damage. Monoclonal IgM gammopathy was identified in all 11 patients, among which 6 were IgM κ, 2 IgG κ and IgM κ bi-clonal, 3 IgM λ. Three patients were diagnosed with Waldenström’s macroglobulinaemia. The anti-MAG-IgM antibody was positive in all 11 cases. After diagnosis, 9 patients received combination chemotherapy including rituximab or rituximab treatment alone. The monoclonal IgM level declined significantly in 7 patients. The neuropathy was stable or improved.
Conclusions:Anti-MAG antibody positive IgM-PN is a rare M protein related disease. In peripheral neuropathy with undetermined etiology, we suggest to screen M protein and anti-MAG antibody. Chemotherapy including rituximab or rituximab alone is recommended as first-line therapy.
