• Internal medicine · Dec 2021

    Case Reports

    High-grade Primary Central Nervous System Lymphomatoid Granulomatosis: Successful Rituximab Monotherapy.

    • Sakiko Harada, Jun Ando, Miki Ando, Hideaki Nitta, Tadaaki Inano, Yusuke Hirasawa, Yoshiki Furukawa, Shintaro Kinoshita, Akihide Kondo, Koichi Ohshima, and Norio Komatsu.
    • Department of Hematology, Juntendo University School of Medicine, Japan.
    • Intern. Med. 2021 Dec 1; 60 (23): 3795-3799.

    AbstractThe primary central nervous system (CNS) presentation of lymphomatoid granulomatosis (LYG) is rare, and no standard therapy for LYG with primary CNS symptoms exists. CNS-LYG patients usually survive for only less than a year from diagnosis. This is the first report of high-grade primary CNS-LYG with monoclonality that was successfully treated with rituximab monotherapy, resulting in a durable remission for more than 1 year in a 66-year-old woman with pemphigus vulgaris who was also on immunosuppressive therapy.

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