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Value of Nerve Biopsy in Patients With Latent Malignant Hemopathy and Peripheral Neuropathy: A Case Series

机译:潜在恶性血液病和周围神经病变患者神经活检的价值:一个病例系列

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Abstract: Hematological malignancies include several diseases that may affect the peripheral nervous system (PNS) through various mechanisms. A common and challenging situation is represented by the occurrence of an active peripheral neuropathy in a patient with a supposed inactive hematological disorder. We report clinical, electrophysiological, biological, and pathological data of 8 patients with latent malignant hemopathies (most were considered in remission): B-cell chronic lymphocytic leukemia in 3 patients, B-cell lymphoma in 1 patient, low-grade non-Hodgkin's lymphoma in 1 patient, Waldenstr?m's macroglobulinemia in 1 patient, smoldering multiple myeloma in 1 patient, and monoclonal gammopathy of undetermined significance in 1 patient. In all these cases, the nerve biopsy (NB) helped to diagnose the hematological relapse or detect a pathological mechanism linked to the hematological disorder: epineurial lymphocytic infiltration in 5 patients (including one with antimyelin-associated glycoprotein antibodies), cryoglobulin deposits in 1 patient, chronic inflammatory demyelinating polyneuropathy in 1 patient, and necrotizing vasculitis in 1 patient. In each case, pathological findings were crucial to select the adequate treatment, leading to an improvement in the neurological and biological manifestations. These observations illustrate the value of NB and the need for active collaboration between neurologists and hematologists in such cases.
机译:摘要:血液系统恶性肿瘤包括几种可能通过各种机制影响周围神经系统(PNS)的疾病。常见且具有挑战性的情况是,在假定的血液学失调患者中发生活动性周围神经病变。我们报告了8例潜在恶性血液病患者的临床,电生理,生物学和病理学数据(大多数患者已考虑缓解):3例患者的B细胞慢性淋巴细胞白血病,1例患者的B细胞淋巴瘤,低度非霍奇金淋巴瘤淋巴瘤1例,华氏巨球蛋白血症1例,闷热多发性骨髓瘤1例,单克隆丙种球蛋白病1例意义不明。在所有这些情况下,神经活检(NB)有助于诊断血液学复发或发现与血液学疾病有关的病理机制:5例患者的肾小管淋巴细胞浸润(包括1例具有抗髓磷脂相关糖蛋白抗体的抗体),1例患者的冷球蛋白沉积物,慢性炎症性脱髓鞘性多发性神经病1例,坏死性血管炎1例。在每种情况下,病理结果对于选择适当的治疗都是至关重要的,从而导致神经和生物学表现的改善。这些观察结果说明了NB的价值以及在这种情况下神经病学家和血液学家之间积极合作的需要。

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