Enlargement of deep medullary veins during the early clinical course of Sturge-Weber syndrome

Abstract

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. 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 .