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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Detalles Bibliográficos
Autores principales: Harada, Sakiko, Ando, Jun, Ando, Miki, Nitta, Hideaki, Inano, Tadaaki, Hirasawa, Yusuke, Furukawa, Yoshiki, Kinoshita, Shintaro, Kondo, Akihide, Ohshima, Koichi, Komatsu, Norio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2021
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8710380/
https://www.ncbi.nlm.nih.gov/pubmed/34121006
http://dx.doi.org/10.2169/internalmedicine.7232-21
Descripción
Sumario: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.

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