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Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome
Schnitzler syndrome is a rare adult-onset acquired autoinflammatory disorder typically characterized by chronic urticarial rash and immunoglobulin M (IgM) (rarely IgG) monoclonal gammopathy. Its clinical symptoms usually respond well to interleukin-1 blockade therapy, which, however, does not impact...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2022
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9065412/ https://www.ncbi.nlm.nih.gov/pubmed/35514998 http://dx.doi.org/10.3389/fimmu.2022.894464 |
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author | Huang, Yuehua Wang, Yanying Yu, Fan Mao, Xuehan Wang, Bianhong Li, Jingxian Li, Lihong |
author_facet | Huang, Yuehua Wang, Yanying Yu, Fan Mao, Xuehan Wang, Bianhong Li, Jingxian Li, Lihong |
author_sort | Huang, Yuehua |
collection | PubMed |
description | Schnitzler syndrome is a rare adult-onset acquired autoinflammatory disorder typically characterized by chronic urticarial rash and immunoglobulin M (IgM) (rarely IgG) monoclonal gammopathy. Its clinical symptoms usually respond well to interleukin-1 blockade therapy, which, however, does not impact the underlying monoclonal gammopathy. Herein, we described a female patient who presented with urticarial rash, recurrent fevers, and fatigue for 7 years. Laboratory investigations revealed IgMκ monoclonal protein and MYD88 L265P mutation, but no lymphoplasmacytic lymphoma on bone marrow examination. She fulfilled the diagnosis of Schnitzler syndrome and was treated with the Bruton tyrosine kinase inhibitor ibrutinib in combination with prednisone. Her symptoms improved dramatically, and the level of IgMκ monoclonal protein also declined. She tolerated the treatment well. This case highlights the potential therapeutic role of Bruton tyrosine kinase inhibitors in Schnitzler syndrome. |
format | Online Article Text |
id | pubmed-9065412 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-90654122022-05-04 Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome Huang, Yuehua Wang, Yanying Yu, Fan Mao, Xuehan Wang, Bianhong Li, Jingxian Li, Lihong Front Immunol Immunology Schnitzler syndrome is a rare adult-onset acquired autoinflammatory disorder typically characterized by chronic urticarial rash and immunoglobulin M (IgM) (rarely IgG) monoclonal gammopathy. Its clinical symptoms usually respond well to interleukin-1 blockade therapy, which, however, does not impact the underlying monoclonal gammopathy. Herein, we described a female patient who presented with urticarial rash, recurrent fevers, and fatigue for 7 years. Laboratory investigations revealed IgMκ monoclonal protein and MYD88 L265P mutation, but no lymphoplasmacytic lymphoma on bone marrow examination. She fulfilled the diagnosis of Schnitzler syndrome and was treated with the Bruton tyrosine kinase inhibitor ibrutinib in combination with prednisone. Her symptoms improved dramatically, and the level of IgMκ monoclonal protein also declined. She tolerated the treatment well. This case highlights the potential therapeutic role of Bruton tyrosine kinase inhibitors in Schnitzler syndrome. Frontiers Media S.A. 2022-04-20 /pmc/articles/PMC9065412/ /pubmed/35514998 http://dx.doi.org/10.3389/fimmu.2022.894464 Text en Copyright © 2022 Huang, Wang, Yu, Mao, Wang, Li and Li https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Immunology Huang, Yuehua Wang, Yanying Yu, Fan Mao, Xuehan Wang, Bianhong Li, Jingxian Li, Lihong Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome |
title | Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome |
title_full | Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome |
title_fullStr | Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome |
title_full_unstemmed | Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome |
title_short | Case Report: Therapeutic Use of Ibrutinib in a Patient With Schnitzler Syndrome |
title_sort | case report: therapeutic use of ibrutinib in a patient with schnitzler syndrome |
topic | Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9065412/ https://www.ncbi.nlm.nih.gov/pubmed/35514998 http://dx.doi.org/10.3389/fimmu.2022.894464 |
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