Acalabrutinib May Offer a New Therapeutic Approach for Consolidation and Maintenance of Primary CNS Lymphoma with Expression of MYD88 and CD79B Gene Variants: A Case Report and Literature Review of Primary CNS Lymphoma in the BTKi Era

TL;DR

A patient with primary CNS lymphoma, who couldn't complete standard treatment, achieved long-term remission using acalabrutinib, a new potential therapy for this condition.

Contribution

This is the first reported case of acalabrutinib used for consolidation and maintenance in primary CNS lymphoma.

Findings

A patient with PCNSL and MYD88/CD79B gene variants achieved 18-month remission with acalabrutinib.

BTK inhibitors are emerging as salvage and first-line treatments for PCNSL in clinical trials.

This case supports further clinical trial design for BTKi use in PCNSL treatment.

Abstract

We present the case of a patient with primary CNS lymphoma (PCNSL), with MYD88 and CD79B gene variants, who was unable to complete standard induction and consolidation treatment due to toxicity and co-morbidities after three cycles of MATRix. Although he had responded to truncated induction, acalabrutinib, the BTK inhibitor, was used in an attempt to consolidate and maintain his response. He has an ongoing remission at 18 months of follow-up. Following the case presentation, we provide a review of PCNSL, the evolution of therapy, and how BTK inhibitors are now emerging treatments incorporated into the salvage of relapsed and refractory disease and into first-line treatment in some clinical trials. This is the first reported case in the literature of acalabrutinib use for consolidation and maintenance of PCNSL. We hope this can support clinical trial design for BTKi use in this setting in the future.

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