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首页> 美国卫生研究院文献>Acta Neuropathologica Communications >Nerve pathologic features differentiate POEMS syndrome from CIDP
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Nerve pathologic features differentiate POEMS syndrome from CIDP

机译：神经病理学特征将POEMS综合征与CIDP区别开来

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The objective of this study is to determine if the nerve pathology in patients with POEMS syndrome is different from CIDP. We hypothesized that nerve biopsies from patients with POEMS syndrome would have more small vessels and axonal degeneration but less inflammation than CIDP.We performed a retrospective analysis of nerve biopsies performed on “classic” CIDP and POEMS cases. Nerve biopsies were blinded and reviewed by two of the authors (EAP, PJBD). Teased fibers, paraffin-embedded sections, semithin sections and immunostains were analyzed. Small endoneurial and epineurial vessels were counted on paraffin sections with smooth muscle actin (SMACTIN) preparation to judge for neovascularization.A total of 61 cases (35-POEMS, 26-CIDP) were included. The POEMS-group had significantly higher axonal degeneration and fewer normal myelinated fibers on teased fiber preparations. The CIDP-group had significantly more endoneurial mononuclear inflammation on paraffin sections and immunostains. Large onion-bulbs were present only in CIDP cases. A significantly higher number of epineurial vessels was present in POEMS biopsies, with a total count of 120 epineurial vessels predicted as best cutoff to differentiate both conditions (77 % specific and 54 % sensitive).In conclusion, nerve biopsy can be helpful in distinguishing POEMS syndrome from CIDP. POEMS syndrome demonstrates more axonal degeneration and epineurial neovascularization whereas CIDP has greater endoneurial inflammation and onion-bulb formation. These findings support the idea that there are differing underlying mechanisms for these disorders, POEMS being related to paraneoplastic vasculopathy associated with angiogenic factors and CIDP related to inflammatory demyelination.
机译：这项研究的目的是确定POEMS综合征患者的神经病理学是否与CIDP不同。我们假设来自POEMS综合征患者的神经活检比CIDP具有更多的小血管和轴突变性，但炎症更少。我们对“经典” CIDP和POEMS病例进行了神经活检的回顾性分析。两名作者（EAP，PJBD）对神经活检进行了盲法检查。分析了取笑的纤维，石蜡包埋的切片，半薄切片和免疫染色。用平滑肌肌动蛋白（SMACTIN）制剂在石蜡切片上计数小神经内膜和肾上腺血管，以判断是否有新生血管形成，包括61例（35-POEMS，26-CIDP）。 POEMS组在戏弄的纤维制品上具有明显更高的轴突变性和较少的正常髓鞘纤维。 CIDP组在石蜡切片和免疫染色上有明显更多的神经内膜单核发炎。仅在CIDP病例中存在大洋葱鳞茎。 POEMS活检中存在大量的肾上腺血管，预计120个肾上腺血管的总数是区分这两种情况的最佳临界值（特异性为77％，敏感性为54％）。总而言之，神经活检可以帮助区分POEMS CIDP综合征。 POEMS综合征表现出更多的轴索变性和海马神经新生血管形成，而CIDP具有更大的神经内膜炎症和洋葱鳞茎形成。这些发现支持了以下观点：这些疾病具有不同的潜在机制，POEMS与与血管生成因子相关的副肿瘤性血管病变和与炎性脱髓鞘相关的CIDP有关。

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