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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.
Acknowledgments
Acknowledgment: The authors thank Cathie Germain, MA, and Cynthia Burnett, BA, for assistance with patient scheduling; Jane Cornett, RN, and Anne Deboard, RN, for performing sedation for imaging; Yang Xuan, BS, for MRI acquisition and preprocessing; and Michael Behen, PhD, for cognitive assessment of the patients.
Footnotes
Supplemental data at Neurology.org
Author contributions: Vinod Pilli: performed image processing, analysis, and interpretation; prepared illustrations; and drafted the manuscript. Harry T. Chugani: involved in study conception and design, patient recruitment and follow-up, data interpretation, and revision of the manuscript. Csaba Juhasz: involved in study conception and design, assisted patient recruitment, supervised data collection and analysis, and revised and finalized the manuscript.
Study funding: NIH (NS041922).
Disclosure: V. Pilli has received salary support from grant 041922 from the National Institute of Neurologic Disorders and Stroke (NINDS). H. Chugani has received research support from NINDS grants NS041922, NS064989, NS079429, NS064033, and NS089659, and Department of Defense grant TS130067. C. Juhász has received research support from NINDS grants NS041922, NS064033, NS089659, and NS065705, and National Cancer Institute grant CA123451. Go to Neurology.org for full disclosures.
- Received April 13, 2016.
- Accepted in final form September 22, 2016.
- © 2016 American Academy of Neurology
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