Case report: Mononeuropathy multiplex of extranodal natural killer/T-cell lymphoma misdiagnosed as systemic vasculitis

Jiayu Shi; Jingwen Niu; Di Wu; Lei Zhang; Yangyu Huang; Hui Zhang; Hongzhi Guan; Mingsheng Liu; Yuzhou Guan

doi:10.3389/fneur.2023.1283874

Case report: Mononeuropathy multiplex of extranodal natural killer/T-cell lymphoma misdiagnosed as systemic vasculitis

Front Neurol. 2023 Nov 21:14:1283874. doi: 10.3389/fneur.2023.1283874. eCollection 2023.

Authors

Jiayu Shi¹, Jingwen Niu¹, Di Wu², Lei Zhang¹, Yangyu Huang¹, Hui Zhang³, Hongzhi Guan¹, Mingsheng Liu¹, Yuzhou Guan¹

Affiliations

¹ Neurology Department, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
² Rheumatology and Immunology Department, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
³ Pathology Department, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.

Abstract

Background: Extranodal NK/T-cell lymphoma (ENKTL) is an aggressive non-Hodgkin lymphoma that typically develops in the upper aerodigestive tract.

Case presentation: We encountered an ENKTL patient who presented with purpura-like rashes and foot drops as initial symptoms and later developed other peripheral nerve involvement. The nerve conduction study of both the motor nerve and the sensory nerve showed axonal damage resembling mononeuropathy multiplex. Although the initial response to steroids was encouraging, the patient's symptoms reappeared and aggravated. A biopsy of the abdominal subcutaneous fat tissue with additional immunohistochemistry revealed neoplastic NK/T lymphocytes.

Conclusion: We reported the first case presented as mononeuropathy multiplex as the initial clinical manifestation in ENKTL patients. Lymphoma should be considered in the diagnosis of atypical mononeuropathy in multiplex patients.

Keywords: axonal neuropathy; biopsy; extranodal NK/T-cell lymphoma; immunohistochemistry; mononeuropathy multiplex.

Publication types

Case Reports

Grants and funding

The author(s) declare that financial support was received for the research, authorship, and/or publication of this article. The research was supported by National High Level Hospital Clinical Research Funding (2022-PUMCH-B-017).