Department of Neurology, Beijng Chaoyang Hospital, Capital Medical University, No. 8 South Gongti Road, Chaoyang District, Beijing, 100020, China.
BMC Neurol. 2023 Sep 1;23(1):314. doi: 10.1186/s12883-023-03310-7.
Chronic lymphoproliferative disorders of natural killer cells (CLPD-NK) is a rare lymphoproliferative disease. Peripheral neuropathy is an unusual symptom of CLPD-NK. We report a case of peripheral neuropathy associated with CLPD-NK and perform a review of literatures.
a 62-year-old woman presented with progressive numbness and weakness in both extremities. Electrophysiological examinations indicated a sensorimotor polyneuropathy. Peripheral blood examination revealed that the number of white blood cells (WBC) and lymphocytes were significantly increased. Flow cytometry analysis identified that 84% of the lymphocytes are NK cells that mainly expressed CD56, combined with variable expression of CD16, CD2, CD7, CD94, granzyme B, perforin, and CD158 but negative for CD3. Sural nerve biopsy revealed that a plethora of NK cells infiltrated into nerve fascicles. On treatment with combined cyclophosphamide and corticosteroids, her symptoms rapidly improved. Moreover, the absolute lymphocyte count and its proportion recovered to normal range after 3 months' treatment.
To the best of our knowledge, this is the first case report of peripheral neuropathy associated with CLPD-NK from Chinese. This rare lymphoproliferative disease should be considered if peripheral neuropathy combines with increased WBC or lymphocytes. Immunosuppressive drugs are the major treatment and most patients can achieve a good prognosis.
自然杀伤细胞慢性淋巴增殖性疾病（CLPD-NK）是一种罕见的淋巴增殖性疾病。周围神经病是 CLPD-NK 的一种不常见症状。我们报告一例与 CLPD-NK 相关的周围神经病，并进行文献复习。
一名 62 岁女性出现四肢进行性麻木和无力。电生理检查提示感觉运动性多发性神经病。外周血检查显示白细胞（WBC）和淋巴细胞数量明显增加。流式细胞术分析表明，84%的淋巴细胞为 NK 细胞，主要表达 CD56，同时表达可变的 CD16、CD2、CD7、CD94、颗粒酶 B、穿孔素和 CD158，但 CD3 阴性。腓肠神经活检显示大量 NK 细胞浸润神经束。接受环磷酰胺和皮质类固醇联合治疗后，她的症状迅速改善。此外，经过 3 个月的治疗，绝对淋巴细胞计数及其比例恢复正常范围。
据我们所知，这是首例来自中国的与 CLPD-NK 相关的周围神经病病例报告。如果周围神经病伴有白细胞或淋巴细胞增多，应考虑这种罕见的淋巴增殖性疾病。免疫抑制药物是主要治疗方法，大多数患者预后良好。
