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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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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container_issue 23
container_start_page 3795
container_title Internal Medicine
container_volume 60
creator Harada, Sakiko
Ando, Jun
Ando, Miki
Nitta, Hideaki
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Kinoshita, Shintaro
Kondo, Akihide
Ohshima, Koichi
Komatsu, Norio
description 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.
doi_str_mv 10.2169/internalmedicine.7232-21
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Med.</addtitle><date>2021-12-01</date><risdate>2021</risdate><volume>60</volume><issue>23</issue><spage>3795</spage><epage>3799</epage><pages>3795-3799</pages><artnum>7232-21</artnum><issn>0918-2918</issn><eissn>1349-7235</eissn><notes>ObjectType-Case Study-2</notes><notes>SourceType-Scholarly Journals-1</notes><notes>ObjectType-Feature-4</notes><notes>content type line 23</notes><notes>ObjectType-Report-1</notes><notes>ObjectType-Article-3</notes><notes>Correspondence to Dr.　Miki Ando, m-ando@juntendo.ac.jp</notes><abstract>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. 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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
title High-grade Primary Central Nervous System Lymphomatoid Granulomatosis: Successful Rituximab Monotherapy
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