Clinical analysis of chronic natural killer cell lymphoproliferative disease associated peripheral neuropathy
10.3760/cma.j.cn113694-20230423-00311
- VernacularTitle: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
- Author:
Jiaxiang GUO
1
;
Hongliang GUO
;
Wenli HU
;
Dachun ZHAO
;
Wen DENG
;
Ming LU
Author Information
1. 首都医科大学附属北京朝阳医院神经内科，北京 100020
- Keywords:
Lymphoproliferative disorder;
Peripheral nerve disease;
Clinical
- From:
Chinese Journal of Neurology
2023;56(11):1278-1284
- CountryChina
- Language:Chinese
-
Abstract:
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.
