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

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 wa...

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Autores principales: Lynch, Ryan C., Advani, Ranjana H.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: S. Karger AG 2017
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5649220/
https://www.ncbi.nlm.nih.gov/pubmed/29070995
http://dx.doi.org/10.1159/000480292
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author Lynch, Ryan C.
Advani, Ranjana H.
author_facet Lynch, Ryan C.
Advani, Ranjana H.
author_sort Lynch, Ryan C.
collection PubMed
description 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.
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spelling pubmed-56492202017-10-25 Dramatic Response with Single-Agent Ibrutinib in Multiply Relapsed Marginal Zone Lymphoma with MYD88(L265P) Mutation Lynch, Ryan C. Advani, Ranjana H. Case Rep Oncol Case Report 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. S. Karger AG 2017-09-12 /pmc/articles/PMC5649220/ /pubmed/29070995 http://dx.doi.org/10.1159/000480292 Text en Copyright © 2017 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission.
spellingShingle Case Report
Lynch, Ryan C.
Advani, Ranjana H.
Dramatic Response with Single-Agent Ibrutinib in Multiply Relapsed Marginal Zone Lymphoma with MYD88(L265P) Mutation
title Dramatic Response with Single-Agent Ibrutinib in Multiply Relapsed Marginal Zone Lymphoma with MYD88(L265P) Mutation
title_full Dramatic Response with Single-Agent Ibrutinib in Multiply Relapsed Marginal Zone Lymphoma with MYD88(L265P) Mutation
title_fullStr Dramatic Response with Single-Agent Ibrutinib in Multiply Relapsed Marginal Zone Lymphoma with MYD88(L265P) Mutation
title_full_unstemmed Dramatic Response with Single-Agent Ibrutinib in Multiply Relapsed Marginal Zone Lymphoma with MYD88(L265P) Mutation
title_short Dramatic Response with Single-Agent Ibrutinib in Multiply Relapsed Marginal Zone Lymphoma with MYD88(L265P) Mutation
title_sort dramatic response with single-agent ibrutinib in multiply relapsed marginal zone lymphoma with myd88(l265p) mutation
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5649220/
https://www.ncbi.nlm.nih.gov/pubmed/29070995
http://dx.doi.org/10.1159/000480292
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