神经淋巴瘤病的临床特点和误诊原因分析

Clinical Features and Misdiagnosed Causes of Patients with Neurolymphomatosis

目的分析神经淋巴瘤病的临床特点及误诊原因。方法回顾性分析2008—2018年曾误诊的4例神经淋巴瘤病的临床资料及误诊原因,并复习相关文献。结果4例均出现肢体乏力或感觉障碍,其中双下肢乏力2例,伴感觉障碍2例;病程中有短期发热2例,发现肝脾肿大2例,腰椎穿刺结果显示:脑脊液蛋白升高3例,发现异型淋巴细胞1例。误诊为Guillain-Barre综合征3例、腰椎间盘突出症1例,误诊时间为5~45 d。4例经腰椎穿刺、骨髓穿刺、淋巴结活组织病理检查,确诊为非霍奇金淋巴瘤3例,NK/T细胞淋巴瘤1例。3例在症状出现后8个月内死亡,1例随访中。结论神经淋巴瘤病中侵犯周围神经多为非霍奇金淋巴瘤,症状一般为对称性肢体乏力,易误诊Guillain-Barre综合征,出现神经系统症状后病死率高。对出现对称性肢体乏力,尤其是伴有发热、肝脾增大的患者要注意排查淋巴瘤的可能。

Objective To analyze clinical features and misdiagnosed causes of patients with neurolymphomatosis.Methods Clinical data and misdiagnosed causes of 4 patients with neurolymphomatosis from 2008 and 2018 were retrospectively analyzed,and related literature was reviewed.Results All 4 patients had limb anergy or sensory disturbances,among whom 2 patients had anergy in both lower extremities,and 2 patients had sensory disturbances;2 patients had brief fever during course of the disease,and 2 patients had hepatosplenomegaly.Results of lumbar puncture showed that there were 3 patients with elevated cerebrospinal fluid protein,and 1 patient with atypical lymphoma.Three patients were misdiagnosed as having Guillain-Barre syndrome,and 1 patient as having lumbar disc protrusion.Misdiagnosed duration was 5-45 d.Of the 4 patients,after examinations of lumbar puncture,bone marrow puncture and lymph node biopsy,3 patients were confirmed as having non-Hodgkin lymphoma,and 1 as having NK/T cell lymphoma.Three patients died within 8 months after the onset of symptoms,and 1 patient was under follow-up.Conclusion Neurolymphomatosis with peripheral nervous invasion is mostly non-Hodgkin lymphoma with commonly symmetrical anergy of limbs,and it is easily misdiagnosed as Guillain-Barre syndrome.The death rate is high after having neurological symptoms.Patients with symmetrical anergy of limbs,especially those with fever and hepatosplenomegaly,should be paid more attention to the possibility of lymphoma.