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    We herein report a 36-year-old man with repeated necrotizing lymphadenitis due to MEFV gene mutations. The patient's chief complaints were a fever and painful cervical lymphadenopathy. We diagnosed him with necrotizing lymphadenitis based on the pathological findings of the lymph nodes and the exclusion of other differential diseases. The same episode recurred four times. We speculated the involvement of autoinflammatory backgrounds and detected MEFV gene mutations of E148Q (homo), P369S, and R408Q. Considering the elevation of interleukin-18, these mutations probably played roles in the repeated necrotizing lymphadenitis.

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    Yuta Yamamura, Kengo Furuichi, Tadashi Toyama, Megumi Oshima, Hisayuki Ogura, Koichi Sato, Shiori Nakagawa, Taro Miyagawa, Shinji Kitajima, Akinori Hara, Yasunori Iwata, Norihiko Sakai, Miho Shimizu, Hiroko Ikeda, Tomoko Toma, Kazuya Takasawa, Akihiro Yachie, Takashi Wada. Repeated Necrotizing Lymphadenitis with MEFV Gene Mutations. Internal medicine (Tokyo, Japan). 2022 Apr 01;61(7):1105-1110

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    PMID: 34511567

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