Julien J, Vital C, Vallat J M, Lagueny A, Ferrer X, Deminiere C, Leboutet M J, Effroy C
Ann Neurol. 1984 Apr;15(4):395-9. doi: 10.1002/ana.410150415.
A 59-year-old man developed a sensorimotor neuropathy of the upper and lower limbs, associated with a biclonal gammopathy, within the space of a few months. Each of two paraproteins was coupled with a distinct IgM kappa IgG lambda light chain. Examination of a nerve biopsy specimen by electron microscopy revealed a demyelinative process with a widening of the interlaminar space in the myelin sheath, as well as deposits of amyloid substance between nerve fibers. Direct immunofluorescence revealed the presence of IgM and of the kappa light chain in certain Schwann cells, while the lambda IgG was fixed to the amyloid deposits. Immunoperoxidase histochemistry showed a positive reaction in normal human nerve tissue to the immune serum IgM and kappa light chain. The findings suggest that the widening of the interlaminar space of the diseased myelin corresponds to an active fixation of immunoglobulin on the sheath of the Schwann cell. The presence of two light chains in this patient's gammopathy caused a dual pathology: the kappa chain, a demyelinative neuropathy, and the lambda chain, a primary amyloidosis, with deposits in the peripheral nerve and in the kidney.
一名59岁男性在几个月内出现了上下肢感觉运动性神经病变，并伴有双克隆丙种球蛋白病。两种副蛋白各自与一种独特的IgM κ IgG λ轻链结合。通过电子显微镜检查神经活检标本发现有脱髓鞘过程，髓鞘层间间隙增宽，以及神经纤维之间有淀粉样物质沉积。直接免疫荧光显示在某些施万细胞中有IgM和κ轻链存在，而λ IgG则固定在淀粉样沉积物上。免疫过氧化物酶组织化学显示正常人神经组织对免疫血清IgM和κ轻链呈阳性反应。这些发现表明，患病髓鞘层间间隙增宽对应于免疫球蛋白在施万细胞鞘上的活跃固定。该患者丙种球蛋白病中两种轻链的存在导致了双重病理改变：κ链导致脱髓鞘性神经病变，λ链导致原发性淀粉样变性，沉积物存在于周围神经和肾脏中。
