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Therapeutic improvements are required for primary CNS lymphoma (PCNSL) and secondary CNS lymphoma. PCNSLs are predominantly diffuse large B-cell lymphoma (DLBCL) classified in the non-germinal center (non-GC) subgroup.1 The role of B-cell receptor (BCR) signaling to continuously activate the NF-κB pathway is well-established in non-GC DLBCL.2 Mutations of MYD88, CD79B, and TBL1XR1, genes involved in the NF-κB pathway, are frequently encountered in PCNSL.3 Ibrutinib, an inhibitor of BCR signaling, led to an objective response rate of 50% in patients with relapsed or refractory systemic non-GC DLBCL.4 As a small molecule (MW = 440), with promising CNS distribution,5 ibrutinib represents a potential treatment for PCNSL. We report a retrospective case series of patients with relapsed and refractory CNS lymphoma treated with ibrutinib.
Footnotes
Supplemental data at Neurology.org
Author contributions: Dr. Chamoun: acquisition of data, analysis and interpretation, critical revision of the manuscript for important intellectual content. Drs. Choquet, Boyle, Houillier, Larrieu-Ciron, Al Jijakli, Delrieu, Delwail, Morschhauser, and Hoang-Xuan: acquisition of data, critical revision of the manuscript for important intellectual content. Dr. Soussain: study concept and design, acquisition of data, analysis and interpretation, critical revision of the manuscript for important intellectual content, study supervision.
Study funding: No targeted funding reported.
Disclosure: K. Chamoun, S. Choquet, E. Boyle, C. Houillier, D. Larrieu-Ciron, A. Al Jijakli, V. Delrieu, V. Delwail, F. Morschhauser, and K. Hoang-Xuan report no disclosures relevant to the manuscript. C. Soussain received research support from Pharmacyclics. Go to Neurology.org for full disclosures.
- Received May 7, 2016.
- Accepted in final form September 21, 2016.
- © 2016 American Academy of Neurology
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