神经淋巴瘤病的研究进展

神经淋巴瘤病(neurolymphomatosis, NL)是一种原发或继发于非霍奇金淋巴瘤(non-Hodgkin's lymphoma, NHL)和白血病的恶性疾病, 主要由淋巴瘤细胞直接浸润周围神经引起, 发病率较低。根据浸润神经不同, 其临床表现多样, 多以受累神经丛/根支配肌肉的肌力减退为主要表现, 常被误诊为吉兰-巴雷综合征、慢性炎性脱髓鞘性多发性神经病(chronic inflammatory demyelinating polyneuropathy, CIDP)等多发性神经病变, 结合丙种免疫球蛋白或类固醇治疗后症状短暂改善, 但疾病仍快速进展, 从而引起临床上的关注。本文旨在从发病机制、临床表现、鉴别诊断、诊断方法、治疗及预后等5个方面对NL近年来的临床报告和研究进行综述, 为临床诊治提供理论依据。 

Progress in research on neurolymphomatosis

Neurolymphomatosis is a malignant disease that occurs primary or secondary to non-Hodgkin's lymphoma and leukemia.It is mainly caused by lymphoma cells directly infiltrating peripheral nerves, and the incidence is low.The clinical manifestations vary according to the involved nerves and mainly present as decreased strength in the muscles innervated by the affected plexus/root.It is often misdiagnosed as a polyneuropathy such as Guillain-Barre syndrome or chronic inflammatory demyelinating polyneuropathy.With combined treatment using immunoglobulins or steroids, the symptoms are temporarily improved but the disease still progresses rapidly, eventually requiring the attention of a physician.This paper will discuss clinical reports and research on neurolymphomatosis in recent years from the aspects of pathogenesis, clinical manifestations, differential diagnosis, diagnostic methods, treatment, and prognosis, and will provide a theoretical basis for clinical diagnosis and treatment.