神经意义的单克隆免疫球蛋白血症诊治进展

Progress of diagnosis and treatment of monoclonal gammopathy of neural significance

神经意义的单克隆免疫球蛋白血症(MGNS)属于临床意义的单克隆免疫球蛋白血症范畴,是主要发生于周围神经的较早期病变,不足以诊断多发性骨髓瘤或淋巴瘤。MGNS需要与POEMS综合征、轻链型淀粉样变性等所致的神经病变进行鉴别;必要时可行神经活组织检查明确外周神经症状与淋巴浆细胞病之间存在的关系。MGNS治疗上建议给予包括静脉注射丙种球蛋白、血浆置换等处理,以及CD20单抗等抗淋巴浆细胞肿瘤靶向治疗。早期识别和干预MGNS,并进行多学科合作,有助于降低恶变风险及致残率。

Monoclonal gammopathy of neural significance(MGNS)belongs to the category of monoclonal gammopathy of clinically significance.It is an early-stage disease that mainly occurs in peripheral nerves and is not sufficient for the diagnosis of multiple myeloma or lymphoma.MGNS needs to be differentiated from neuropathies due to POEMS syndrome and light-chain amyloidosis;if necessary,nerve biopsy can be performed to clarify the relationship between peripheral nerve symptoms and lymphoplasmacytic disease.Treatment of MGNS is recommended to give intravenous gammaglobulin,plasma exchange and targeted anti-lymphoplasmacytic tumour therapy such as CD20 monoclonal antibody.Early recognition and intervention of MGNS,with multidisciplinary cooperation,will help to reduce the risk of malignancy and the incidence of disability.