Eurelings M, Notermans N C, Wokke J H J, Bosboom W M J, Van den Berg L H
Department of Neurology, the Rudolf Magnus Institute of Neuroscience, University Medical Center Utrecht, G03.228, PO Box 85500, 3508 GA Utrecht, The Netherlands.
Acta Neuropathol. 2002 Feb;103(2):107-14. doi: 10.1007/s004010100437. Epub 2001 Nov 22.
The clinical presentation of polyneuropathy associated with monoclonal gammopathy is heterogeneous. As T cells in sural nerve biopsy specimens may represent a marker of inflammation, we analyzed whether the presence of sural nerve T cells in patients with demyelinating polyneuropathy associated with monoclonal gammopathy may help to define a specific clinical entity. Using immunohistochemical analysis we investigated the number and distribution of sural nerve T cells in 18 patients with polyneuropathy associated with IgM monoclonal gammopathy (including 14 with antibodies to the myelin-associated glycoprotein) and 7 with IgG monoclonal gammopathy, and compared them with sural nerves of 23 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 15 patients with chronic idiopathic axonal polyneuropathy (CIAP), and 10 normal controls. Six patients with polyneuropathy associated with monoclonal gammopathy had increased T cell densities compared with CIAP patients and normal controls. No differences were found in distribution or phenotype of the T cells. T cell densities in patients with IgM monoclonal gammopathy were significantly lower than in patients with IgG monoclonal gammopathy or with CIDP. Increased sural nerve T cells were significantly associated with a subset of patients who had a more progressive disease course and more pronounced weakness. Increased sural nerve T cells were found significantly more often in patients with a monoclonal gammopathy of the IgG isotype, which was frequently associated with hematological malignancy.
与单克隆丙种球蛋白病相关的多发性神经病的临床表现具有异质性。由于腓肠神经活检标本中的T细胞可能代表炎症标志物,我们分析了在与单克隆丙种球蛋白病相关的脱髓鞘性多发性神经病患者中,腓肠神经T细胞的存在是否有助于定义一种特定的临床实体。我们采用免疫组织化学分析方法,研究了18例与IgM单克隆丙种球蛋白病相关的多发性神经病患者(包括14例具有髓鞘相关糖蛋白抗体的患者)和7例与IgG单克隆丙种球蛋白病相关的多发性神经病患者腓肠神经T细胞的数量和分布,并将其与23例慢性炎症性脱髓鞘性多发性神经病(CIDP)患者、15例慢性特发性轴索性多发性神经病(CIAP)患者的腓肠神经以及10例正常对照者的腓肠神经进行比较。与CIAP患者和正常对照相比,6例与单克隆丙种球蛋白病相关的多发性神经病患者的T细胞密度增加。在T细胞的分布或表型方面未发现差异。IgM单克隆丙种球蛋白病患者的T细胞密度显著低于IgG单克隆丙种球蛋白病患者或CIDP患者。腓肠神经T细胞增加与疾病进展更快、肌无力更明显的一部分患者显著相关。在IgG同种型单克隆丙种球蛋白病患者中,腓肠神经T细胞增加的情况明显更常见,而IgG同种型单克隆丙种球蛋白病常与血液系统恶性肿瘤相关。