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

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 1year in a 66-year-old woman with pemphigus vulgaris who was also on immunosuppressive therapy.

Rituximab Monotherapy for Grade 2-3 Lymphomatoid Granulomatosis with Central Nervous System Involvement in a Patient Receiving Methotrexate for Rheumatoid Arthritis

“…The use of rituximab monotherapy has not been reported previously, and a combination of rituximab with chemotherapy is selected for MTX-LPD with CNS lesions ( 16 ). However, rituximab monotherapy was reported to be effective for some patients with LYG, although these cases involved patients with LYG that was unrelated to MTX-LPD ( 17 , 18 ). Due to the reported efficacy of rituximab monotherapy against LYG and our patient's frailty due to RA, our patient was treated with rituximab monotherapy rather than high-dose MTX-based chemotherapy.…”