霍奇金淋巴瘤发病的副肿瘤神经综合征

E. S. Pavlyuchenko, Павлюченко Елена Сергеевна, A. A. Mirsaitov, Мирсаитов Александр Андреевич, Mariya N. Diakonova, Дьяконова Мария Николаевна, E. Karev, Карев Егор Александрович
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摘要

霍奇金淋巴瘤是一种b细胞克隆性增殖和高水平反应性炎症微环境的恶性疾病。临床主要表现为淋巴结病变和中毒症状。神经系统症状通常是神经结构受压或肿瘤浸润的结果。中枢神经系统的主要损害发生在所有HL病例的0.2%至0.5%之间。副肿瘤神经系统综合征是一组罕见的以肿瘤过程为背景的神经系统疾病(10000例患者中平均1例)。其病理生理机制是产生针对肿瘤细胞和神经细胞的抗体。这些抗体被称为神经自身抗体。使诊断更加困难的标志是肿瘤神经自身抗体很少出现在淋巴瘤患者中,除非在边缘脑炎或副肿瘤小脑变性患者中出现抗tr和抗mglur1抗体。本文报道两例霍奇金淋巴瘤发病时伴有PNS的病例。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Paraneoplastic neurological syndrome in onset of Hodgkin lymphoma
Hodgkin lymphoma is a malignant disease with clonal proliferation of B-cells and high-level reactive inflammatory microenvironment. The main clinical sings are lymphadenopathy and toxic symptoms. Neurological symptoms as usual can be a result of compression or tumor infiltration of nervous structures. The primary damage of CNS occurs from 0,2% to 0,5% of all cases HL. Paraneoplastic neurological syndrome is a group of rare (an average 1 case on 10000 patients) neurological disorders against the background of oncological process. The pathophysiologic mechanism is due to production of antibody which is both to tumor cells and nerve cells. These antibodies are called onconeural autoantibodies. The hallmark which make diagnostics harder is the fact that onconeural autoantibodies rare take place in patients with lymphomas unless anti-Tr and anti-mGluR1 in patients with limbic encephalitis or paraneoplastic cerebellar degeneration. There are two case reports about patients with PNS in onset of Hodgkin lymphoma in article.
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