University of Nebraska Medical Center, 988435 Nebraska Medical Center, Omaha, NE, 68198-8435, USA.
Mayo Clinic, Department of Neurology, 200 First Street SW, Rochester, MN, 55905, USA.
Acta Neuropathol Commun. 2016 Oct 31;4(1):116. doi: 10.1186/s40478-016-0389-1.
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.
本研究旨在确定 POEMS 综合征患者的神经病理学是否与 CIDP 不同。我们假设 POEMS 综合征患者的神经活检将具有更多的小血管和轴突变性，但炎症少于 CIDP。
我们对“经典”CIDP 和 POEMS 病例进行了神经活检的回顾性分析。神经活检由两位作者（EAP、PJBD）进行盲法和审查。 teased fibers、石蜡包埋切片、半薄切片和免疫染色进行了分析。用平滑肌肌动蛋白（SMACTIN）制备对石蜡切片上的小神经内膜和神经外膜血管进行计数，以判断新生血管形成。
共纳入 61 例（35 例 POEMS，26 例 CIDP）。POEMS 组在 teased fiber 准备中具有明显更高的轴突变性和更少的正常髓鞘纤维。CIDP 组在石蜡切片和免疫染色上具有明显更多的神经内膜单核炎症。洋葱球仅存在于 CIDP 病例中。POEMS 活检中有明显更多的神经外膜血管，总计数为 120 个神经外膜血管被预测为最佳截断值，以区分两种情况（77%特异性和 54%敏感性）。
总之，神经活检有助于区分 POEMS 综合征和 CIDP。POEMS 综合征表现出更多的轴突变性和神经外膜新生血管形成，而 CIDP 具有更大的神经内膜炎症和洋葱球形成。这些发现支持这样一种观点，即这些疾病的潜在机制不同，POEMS 与与血管生成因子相关的副肿瘤性血管病有关，而 CIDP 与炎症性脱髓鞘有关。
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