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

The 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...

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Bibliographic Details
Published in:Internal Medicine 2021/12/01, Vol.60(23), pp.3795-3799
Main Authors: Harada, Sakiko, Ando, Jun, Ando, Miki, Nitta, Hideaki, Inano, Tadaaki, Hirasawa, Yusuke, Furukawa, Yoshiki, Kinoshita, Shintaro, Kondo, Akihide, Ohshima, Koichi, Komatsu, Norio
Format: Article
Language:English
Subjects:
Case Report
Central Nervous System
Female
Granulomatosis
high-grade lymphomatoid granulomatosis
Humans
Immunosuppressive agents
Internal medicine
Lymphomatoid Granulomatosis - diagnostic imaging
Lymphomatoid Granulomatosis - drug therapy
Nervous system
Pemphigus
pemphigus vulgaris
Remission
Rituximab
Rituximab - therapeutic use
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Summary:The 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.
ISSN:0918-2918
1349-7235
DOI:10.2169/internalmedicine.7232-21