Kamiya-Matsuoka Carlos, Shroff Sheetal, Gildersleeve Kasey, Hormozdi Bahram, Manning John T, Woodman Karin H
Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA; Department of Neurology, The University of Texas Medical Branch at Galveston, Galveston, TX, USA.
Department of Neuro-Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA; Department of Neurology, The University of Texas Medical Branch at Galveston, Galveston, TX, USA.
J Neurol Sci. 2014 Aug 15;343(1-2):144-8. doi: 10.1016/j.jns.2014.05.058. Epub 2014 Jun 2.
Neurolymphomatosis (NL) is a rare clinical entity characterized by infiltration of malignant lymphocytes into the peripheral nervous system. We analyzed the clinicoradiological features, treatments, and outcomes in NL patients.
We identified six patients with NL seen at The University of Texas MD Anderson Cancer Center from 01/2010 to 10/2012. We extracted clinical presentations, imagings, CSF cytology, and electrodiagnostic studies from medical records. One patient had a nerve biopsy. We defined therapy response as clinical improvement of neurological deficits.
The mean age at onset was 57.1 years. Most were predominantly men with non-Hodgkin lymphoma. Positron emission tomography (PET) was positive in five patients. Nerve conduction demonstrated mononeuritis multiplex, plexopathy, demyelination, and axonal polyradiculoneuropathy, whereas electromyography was nonspecific. All patients received systemic chemotherapy, four intrathecal chemotherapy, and three intravenous immunoglobulin, plasma exchange or both. One patient who received intravenous immunoglobulin showed mild neurological improvement. Two patients responded, and the median overall survival was 15 months.
NL is an increasingly recognized complication of NHL and leukemia. A high clinical suspicion is necessary for correct diagnosis. In the present series, patients with leukemia had mononeuritis multiplex, whereas those with lymphoma had plexopathy. Electrodiagnosis and PET scans were useful diagnostic tools. No factors correlated with poorer prognosis. International collaborative studies will help to better determine the risk factors of NL, response to treatment and outcomes.
神经淋巴瘤（NL）是一种罕见的临床病症，其特征为恶性淋巴细胞浸润外周神经系统。我们分析了NL患者的临床放射学特征、治疗方法及预后。
我们确定了2010年1月至2012年10月在德克萨斯大学MD安德森癌症中心就诊的6例NL患者。我们从病历中提取了临床表现、影像学检查、脑脊液细胞学检查及电诊断研究结果。1例患者进行了神经活检。我们将治疗反应定义为神经功能缺损的临床改善。
发病的平均年龄为57.1岁。大多数患者为男性，主要患有非霍奇金淋巴瘤。5例患者的正电子发射断层扫描（PET）呈阳性。神经传导显示多灶性单神经炎、神经丛病、脱髓鞘及轴索性多神经根神经病，而肌电图检查无特异性。所有患者均接受了全身化疗，4例接受了鞘内化疗，3例接受了静脉注射免疫球蛋白、血浆置换或两者联合治疗。1例接受静脉注射免疫球蛋白治疗的患者神经功能有轻度改善。2例患者有反应，中位总生存期为15个月。
NL是一种越来越被认识到的非霍奇金淋巴瘤和白血病的并发症。正确诊断需要高度的临床怀疑。在本系列研究中，白血病患者有多灶性单神经炎，而淋巴瘤患者有神经丛病。电诊断和PET扫描是有用的诊断工具。没有因素与较差的预后相关。国际合作研究将有助于更好地确定NL的危险因素、对治疗的反应及预后。
