慢性NK细胞淋巴增殖性疾病相关周围神经病患者临床分析

Clinical analysis of chronic natural killer cell lymphoproliferative disease associated peripheral neuropathy

目的探讨慢性NK细胞淋巴增殖性疾病(CLPD-NK)相关周围神经病的临床特征、诊断和治疗。方法报道2022年3月17日就诊于北京朝阳医院的1例CLPD-NK相关周围神经病患者的临床资料,并结合国内外文献总结CLPD-NK相关周围神经病的临床、实验室检验、神经活组织检查(活检)、治疗及转归等情况。结果患者女性,62岁,临床表现为四肢无力、麻木,神经电生理检查提示周围神经损伤,脑脊液常规生化未见异常。腓肠神经活检神经束内见淋巴细胞浸润,CD56(+),TIA-1(+)。予泼尼松联合环磷酰胺治疗后,患者症状减轻,白细胞计数下降。查阅文献,包括本例患者,目前共报道CLPD-NK相关周围神经病患者10例,男女各5例,中位年龄64岁,外周血白细胞计数为5.50×10^(9)/L~24.66×10^(9)/L,淋巴细胞计数为1.54×10^(9)/L~32.00×10^(9)/L;3例患者脑脊液蛋白升高,所有患者腓肠神经活检均可见NK细胞浸润。患者的治疗方案以免疫抑制为主,多数患者预后良好。结论CLPD-NK周围神经病临床较为罕见。周围神经活检是其诊断的重要依据。皮质类固醇及免疫抑制剂治疗对该疾病有效。

Objective To investigate the clinical characteristics,diagnosis and treatment of chronic natural killer cell lymphoproliferative disease(CLPD-NK)associated peripheral neuropathy.Methods The clinical data of 1 case of CLPD-NK associated peripheral neuropathy who was hospitalized in Beijing Chaoyang Hospital on March 17,2022 were reported.The clinical characteristics,laboratory examinations,nerve biopsy,treatment and prognosis of CLPD-NK associated peripheral neuropathy were summarized in combination with domestic and foreign literature reports.Results This patient was a 62-year-old woman with progressive weakness and numbness of limbs.Nerve conduction test revealed peripheral nerves injures of limbs.No abnormality was found in a routine biochemistry test of cerebrospinal fluid.Lymphocyte infiltration was found in the nerve bundle of sural nerve with CD56(+),TIA-1(+).After prednisone combined with cyclophosphamide treatment,her weakness and numbness of limbs were relieved,and the white blood cells count decreased.A total of 10 cases of CLPD-NK associated peripheral neuropathy were summarized,including 5 males and 5 females.The median onset age was 64 years.The peripheral blood white cells count was 5.50×10^(9)/L-24.66×10^(9)/L,the lymphocytes count was 1.54×10^(9)/L-32.00×10^(9)/L,and cerebrospinal fluid protein increased in 3 cases.Biopsy of sural nerve showed NK cell infiltration in all patients.Immunosuppression was the main treatment,and most patients had a good prognosis.Conclusions CLPD-NK associated peripheral neuropathy is rare.Peripheral nerve biopsy is the important diagnostic method.Corticosteroids and immunosuppressants are effective for this disease.