Visentin Andrea, Pravato Stefano, Castellani Francesca, Campagnolo Marta, Angotzi Francesco, Cavarretta Chiara Adele, Cellini Alessandro, Ruocco Valeria, Salvalaggio Alessandro, Tedeschi Alessandra, Trentin Livio, Briani Chiara
Hematology and Clinical Immunology Unit, Department of Medicine, University of Padova, 35128 Padova, Italy.
Neurology Unit, Department of Neurosciences, University of Padova, 35128 Padova, Italy.
Cancers (Basel). 2022 Mar 18;14(6):1562. doi: 10.3390/cancers14061562.
Monoclonal gammopathy and peripheral neuropathy are common diseases of elderly patients, and almost 10% of patients with neuropathy of unknown cause have paraprotein. However, growing evidence suggests that several hematological malignancies synthesize and release monoclonal proteins that damage the peripheral nervous system through different mechanisms. The spectrum of the disease varies from mild to rapidly progressive symptoms, sometimes affecting not only sensory nerve fibers, but also motor and autonomic fibers. Therefore, a multidisciplinary approach, mainly between hematologists and neurologists, is recommended in order to establish the correct diagnosis of monoclonal gammopathy of neurological significance and to tailor therapy based on specific genetic mutations. In this review, we summarize the spectrum of monoclonal gammopathies of neurological significance, their distinctive clinical and neurophysiological phenotypes, the most relevant pathophysiological events and new therapeutic approaches.
单克隆丙种球蛋白病和周围神经病是老年患者的常见疾病，几乎10%病因不明的神经病患者存在副蛋白。然而，越来越多的证据表明，几种血液系统恶性肿瘤会合成并释放单克隆蛋白，这些蛋白通过不同机制损害周围神经系统。该疾病的症状范围从轻度到快速进展，有时不仅影响感觉神经纤维，还影响运动和自主神经纤维。因此，建议主要由血液科医生和神经科医生采用多学科方法，以正确诊断具有神经学意义的单克隆丙种球蛋白病，并根据特定基因突变制定治疗方案。在本综述中，我们总结了具有神经学意义的单克隆丙种球蛋白病的范围、其独特的临床和神经生理学表型、最相关的病理生理事件以及新的治疗方法。
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