Vital C, Deminière C, Lagueny A, Bergouignan F X, Pellegrin J L, Doutre M S, Clement A, Beylot J
Department of Neuropathology, Bordeaux II University, France.
Acta Neuropathol. 1988;75(6):605-10. doi: 10.1007/BF00686206.
Essential mixed cryoglobulinemia, which can cause hypersensitivity vasculitis, was observed in five patients with peripheral neuropathy. Three cases presented with multifocal neuropathies and two cases with symmetrical polyneuropathy. One had cryoglobulinemia with IgM monoclonal gammopathy IgG polyclonal gammopathy, and the other four had cryoglobulinemia with polyclonal gammopathy. Biopsies showed perivascular infiltration by mononuclear cells around medium, and mainly small-sized blood vessels. This was observed in the epineurium (five cases) and muscular fragments (three cases). At ultrastructural examination two cases showed severe damage of most myelinated fibers, which presented acute stages of Wallerian-like degeneration, and the three other cases showed a less widespread destruction of myelinated fibers. Most endoneurial capillaries showed swollen endoneurial cells. Myelino-axonal degeneration of myelinated fibers is probably due mainly to the vasculitis always present in the epineurium. This damage was probably worsened by the modifications of endoneurial capillaries. These lesions and their mechanisms are quite different from those observed in cases of cryoglobulinemia with an isolated monoclonal gammopathy.
5例周围神经病患者中观察到可引起超敏性血管炎的原发性混合性冷球蛋白血症。3例表现为多灶性神经病，2例表现为对称性多发性神经病。1例为伴有IgM单克隆丙种球蛋白病及IgG多克隆丙种球蛋白病的冷球蛋白血症，另外4例为伴有多克隆丙种球蛋白病的冷球蛋白血症。活检显示在中、小血管周围有单核细胞血管周围浸润。在神经外膜（5例）和肌肉组织（3例）中观察到这种情况。超微结构检查显示，2例大多数有髓纤维严重受损，呈现华勒氏样变性急性期，另外3例有髓纤维破坏范围较小。大多数神经内膜毛细血管显示神经内膜细胞肿胀。有髓纤维的髓鞘轴索性变性可能主要归因于神经外膜中始终存在的血管炎。神经内膜毛细血管的改变可能会加重这种损伤。这些病变及其机制与孤立性单克隆丙种球蛋白病性冷球蛋白血症病例中观察到的情况有很大不同。
