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两名患有IgM单克隆丙种球蛋白病和多发性神经病患者的炎性脱髓鞘病变。

Inflammatory demyelinating lesions in two patients with IgM monoclonal gammopathy and polyneuropathy.

作者信息

Vital A, Latinville D, Aupy M, Dumas P, Vital C

机构信息

Department of Neuropathology, University of Bordeaux II, France.

出版信息

Neuropathol Appl Neurobiol. 1991 Oct;17(5):415-20. doi: 10.1111/j.1365-2990.1991.tb00741.x.

Abstract

We report two patients with polyneuropathy and IgM monoclonal gammopathy in whom peripheral nerve biopsy showed the widening of myelin lamellae which is characteristic of IgM paraproteinaemic neuropathy. Moreover, certain myelinated fibres were invaded by histiocytes overloaded with myelin debris, and in some instances elongated macrophage processes could be seen peeling away the myelin lamellae. The latter ultrastructural features are characteristic of inflammatory demyelinating polyneuropathies in both human and experimental pathology. Such an association has not been reported to date in human pathology, but could explain the prevalence of inflammatory demyelinating lesions in experimental models of IgM paraproteinaemic neuropathy. These two cases seem to bridge the gap between inflammatory demyelinating polyneuropathies and polyneuropathies associated with IgM monoclonal gammopathy.

摘要

我们报告了两名患有多神经病和IgM单克隆丙种球蛋白病的患者,其周围神经活检显示髓鞘板层增宽,这是IgM副蛋白血症性神经病的特征。此外,某些有髓纤维被充满髓鞘碎片的组织细胞侵入,在某些情况下,可以看到细长的巨噬细胞突起正在剥脱髓鞘板层。后一种超微结构特征在人类和实验病理学中都是炎性脱髓鞘性多神经病的特征。这种关联在人类病理学中迄今尚未见报道,但可以解释IgM副蛋白血症性神经病实验模型中炎性脱髓鞘性病变的发生率。这两例病例似乎弥合了炎性脱髓鞘性多神经病与与IgM单克隆丙种球蛋白病相关的多神经病之间的差距。

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