高级别原发性中枢神经系统淋巴瘤样肉芽肿病：利妥昔单抗单药治疗成功。

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

机构信息

Department of Hematology, Juntendo University School of Medicine, Japan.

Department of Transfusion Medicine and Stem Cell Regulation, Juntendo University School of Medicine, Japan.

出版信息

Intern Med. 2021 Dec 1;60(23):3795-3799. doi: 10.2169/internalmedicine.7232-21. Epub 2021 Jun 12.

DOI:10.2169/internalmedicine.7232-21

PMID:34121006

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8710380/

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. 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.

摘要

原发性中枢神经系统（CNS）表现的淋巴样肉芽肿病（LYG）较为罕见，且针对原发性 CNS 症状的 LYG 也没有标准的治疗方法。CNS-LYG 患者从诊断到死亡的平均生存时间不足 1 年。这是首例成功应用利妥昔单抗单药治疗的高级别原发性 CNS-LYG 伴单克隆性的报道，一名 66 岁患有寻常型天疱疮且正在接受免疫抑制治疗的女性患者，在接受利妥昔单抗单药治疗后获得了 1 年以上的持久缓解。