Dramatic Response with Single-Agent Ibrutinib in Multiply Relapsed Marginal Zone Lymphoma with MYD88L265P Mutation

Ryan C Lynch; Ranjana H Advani

doi:10.1159/000480292

Dramatic Response with Single-Agent Ibrutinib in Multiply Relapsed Marginal Zone Lymphoma with MYD88^L265P Mutation

Case Rep Oncol. 2017 Sep 12;10(3):813-818. doi: 10.1159/000480292. eCollection 2017 Sep-Dec.

Authors

Ryan C Lynch¹, Ranjana H Advani¹

Affiliation

¹ Division of Oncology, Department of Medicine, Stanford University School of Medicine, Stanford, California, USA.

Abstract

The B-cell receptor signaling pathway is important in the lymphomagenesis of many lymphomas, including marginal zone lymphoma (MZL). Herein we describe a case of extranodal MZL refractory to multiple lines of therapy. The presence of an IgM paraprotein prompted further evaluation, and the patient was found to have an MYD88^L265P mutation. Treatment with ibrutinib led to a dramatic response with prompt resolution of symptoms and significant improvement in measurable sites of disease. The excellent response to ibrutinib in our patient with MYD88^L265P-mutated refractory MZL supports a biological rationale for its use.

Keywords: Marginal zone lymphoma; Targeted therapy.

Publication types

Case Reports