Harbs H, Arfmann M, Frick E, Hörmann C, Wurster U, Patzold U, Stark E, Deicher H
J Neurol. 1985;232(1):43-8. doi: 10.1007/BF00314040.
Sera of 23 patients with Waldenström's macroglobulinaemia and six monoclonal IgM paraproteins, which had been isolated from these sera, were examined for reactivity against peripheral nerve tissue. Of these 23 patients, 12 had clinical signs of peripheral polyneuropathy (PN). Using an indirect immunofluorescence method, all sera and monoclonal IgM preparations reacted with peripheral nerve structures, displaying a distinct granular fluorescence pattern with anti-IgM sera. The Waldenström sera reacted mainly with structures at the border of the myelin sheath, as well as between myelin and axon, and occasionally with the axon itself. There was no difference between sera of patients with PN and those without. Negative results were obtained in a complement fixation assay. Of the 23 sera, 15 reacted in an antibody-dependent lymphocyte-mediated cytotoxicity reaction (ADLC) with peripheral nerve myelin, and to a much lesser extent with myelin basic protein from CNS. Five of the six isolated monoclonal IgM preparations also gave positive ADLC reactions. These results constitute additional evidence for an immunological mechanism in the pathogenesis of PN in Waldenström's macroglobulinaemia.
对23例华氏巨球蛋白血症患者的血清以及从这些血清中分离出的6种单克隆IgM副蛋白进行检测,观察其对外周神经组织的反应性。这23例患者中,12例有外周多发性神经病(PN)的临床症状。采用间接免疫荧光法,所有血清和单克隆IgM制剂均与外周神经结构发生反应,用抗IgM血清显示出明显的颗粒状荧光模式。华氏巨球蛋白血症血清主要与髓鞘边界以及髓鞘与轴突之间的结构发生反应,偶尔也与轴突本身发生反应。有PN的患者血清与无PN的患者血清之间没有差异。补体结合试验结果为阴性。23份血清中,15份在抗体依赖性淋巴细胞介导的细胞毒性反应(ADLC)中与外周神经髓鞘发生反应,与中枢神经系统髓鞘碱性蛋白的反应程度要小得多。6种分离出的单克隆IgM制剂中有5种也给出了阳性ADLC反应。这些结果为华氏巨球蛋白血症中PN发病机制的免疫机制提供了更多证据。