Spectrum of paraneoplastic disease associated with lymphoma.
Spectrum of paraneoplastic disease associated with lymphoma.
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DOI：
10.1212/wnl.0b013e31820d62eb
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发表时间：
2011-02-22
期刊：
影响因子：
9.9
通讯作者：
Giometto, B
中科院分区：
文献类型：
--
作者：
Briani, C;Vitaliani, R;Giometto, B
目的：为了确定受副肿瘤性神经综合征（PNS）和淋巴瘤影响的患者的频率和临床及免疫学特征，对符合从欧盟委员会资助的PNS Euronetwork组数据库中收集的与淋巴瘤相关的PNS标准的患者进行分析。分析了53例霍奇金淋巴瘤（HL）（24例患者，平均年龄51岁，范围16-84）或非霍奇金淋巴瘤（NHL）（29例患者，平均年龄64岁，范围31-82）和PNS患者。最常见的相关PNS是副肿瘤性小脑变性，存在于21例病例中，HL中的患病率较高（16/24例）。NHL以周围神经系统（主要为脱髓鞘性多发性神经根病）和运动神经元受累多见。肿瘤神经抗体在副肿瘤性小脑变性患者中更常见，最常见的是针对Tr抗原。50%的PNS和HL患者对化疗有反应，而PNS和NHL患者的神经功能改善较少（24%）。两组的生存率都很好。总的来说，10/53例患者最终死亡，只有2例（1例HL，1例NHL）死于PNS。结论：淋巴瘤患者的PNS是相对罕见的。主要与抗Tr抗体相关的副肿瘤性小脑变性在HL和NHL中更常见，其次是NHL患者的运动神经元疾病。周围神经系统的受累是异质性的，NHL患者多神经根神经炎的患病率较高。
OBJECTIVE: To define the frequency and clinical and immunologic characteristics of patients affected by paraneoplastic neurologic syndromes (PNS) and lymphoma.METHODS: Patients fulfilling the criteria for PNS associated with lymphoma collected from the European Commission-funded PNS Euronetwork group database were analyzed.RESULTS: Fifty-three patients with Hodgkin lymphoma (HL) (24 patients, mean age 51, range 16-84) or non-Hodgkin lymphoma (NHL) (29 patients, mean age 64, range 31-82) and PNS were analyzed. The most commonly associated PNS was paraneoplastic cerebellar degeneration, present in 21 cases, with a higher prevalence in HL (16/24 cases). Peripheral nervous system (mainly demyelinating polyradiculopathies) and motor neuron involvement were more common in NHL. Onconeural antibodies were more frequent in patients with paraneoplastic cerebellar degeneration, most commonly against the Tr antigen. Fifty percent of the patients with PNS and HL responded to chemotherapy, whereas neurologic improvement was less frequent (24%) in patients with PNS and NHL. In both groups, the survival rate was good. Overall, 10 out of 53 patients eventually died, with only 2 patients (1 with HL, 1 with NHL) dying from PNS.CONCLUSIONS: PNS in patients with lymphoma are relatively rare. Paraneoplastic cerebellar degeneration, mainly associated with anti-Tr antibodies, is more prevalent in HL and NHL, followed in our study by motor neuron disease in patients with NHL. Involvement of the peripheral nervous system is heterogeneous, with a prevalence of polyradiculoneuritis in patients with NHL.