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A first case of successful using of ibrutinib in treating paraneoplastic pemphigus related bronchiolitis obliterans concurrent with CLL

Paraneoplastic pemphigus (PNP) is a rare life-threatening disease which always associated with an underlying neoplasm. Tumor-related PNP most commonly precedes the detection of a hematological malignancy, with some cases seen during disease remission following cytotoxic drug therapy or radiotherapy....

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Autores principales: Chen, Can, Xu, Ying, Yu, Jingdi, Qian, Shenxian, Xie, Yaping
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10056216/
https://www.ncbi.nlm.nih.gov/pubmed/37007770
http://dx.doi.org/10.3389/fmed.2023.1132535
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author Chen, Can
Xu, Ying
Yu, Jingdi
Qian, Shenxian
Xie, Yaping
author_facet Chen, Can
Xu, Ying
Yu, Jingdi
Qian, Shenxian
Xie, Yaping
author_sort Chen, Can
collection PubMed
description Paraneoplastic pemphigus (PNP) is a rare life-threatening disease which always associated with an underlying neoplasm. Tumor-related PNP most commonly precedes the detection of a hematological malignancy, with some cases seen during disease remission following cytotoxic drug therapy or radiotherapy. The lung is the most frequently-involved site in PNP, second only to the eyes, and involvement is seen in 59.2% to 92.8% of PNP cases. Bronchiolitis obliterans (BO) is the end stage of respiratory involvement and is regarded as life-threatening. The key point in treatment of PNP is to control the associated underlying hematologic neoplasia. High-dose systemic corticosteroids combined with other immunosuppressants are considered the first line of treatment. Other therapies that have shown beneficial effects include plasmapheresis, intravenous immunogloblin (IVIG), and more recently, daclizumab, alemtuzumab, and rituximab. There is no effective treatment for BO with PNP, and suppression of the cellular immune response may be necessary. Patients with PNP-BO associated with lymphoma mostly die within approximately 1 year. Herein, we reported a patient who diagnosed with PNP-BO concurrent with chronic lymphocytic leukemia. He was successful treated with ibrutinib and had achieved the longest survival which suggested that ibrutinib may be the best treatment choice for such patient.
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spelling pubmed-100562162023-03-30 A first case of successful using of ibrutinib in treating paraneoplastic pemphigus related bronchiolitis obliterans concurrent with CLL Chen, Can Xu, Ying Yu, Jingdi Qian, Shenxian Xie, Yaping Front Med (Lausanne) Medicine Paraneoplastic pemphigus (PNP) is a rare life-threatening disease which always associated with an underlying neoplasm. Tumor-related PNP most commonly precedes the detection of a hematological malignancy, with some cases seen during disease remission following cytotoxic drug therapy or radiotherapy. The lung is the most frequently-involved site in PNP, second only to the eyes, and involvement is seen in 59.2% to 92.8% of PNP cases. Bronchiolitis obliterans (BO) is the end stage of respiratory involvement and is regarded as life-threatening. The key point in treatment of PNP is to control the associated underlying hematologic neoplasia. High-dose systemic corticosteroids combined with other immunosuppressants are considered the first line of treatment. Other therapies that have shown beneficial effects include plasmapheresis, intravenous immunogloblin (IVIG), and more recently, daclizumab, alemtuzumab, and rituximab. There is no effective treatment for BO with PNP, and suppression of the cellular immune response may be necessary. Patients with PNP-BO associated with lymphoma mostly die within approximately 1 year. Herein, we reported a patient who diagnosed with PNP-BO concurrent with chronic lymphocytic leukemia. He was successful treated with ibrutinib and had achieved the longest survival which suggested that ibrutinib may be the best treatment choice for such patient. Frontiers Media S.A. 2023-03-15 /pmc/articles/PMC10056216/ /pubmed/37007770 http://dx.doi.org/10.3389/fmed.2023.1132535 Text en Copyright © 2023 Chen, Xu, Yu, Qian and Xie. 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 Medicine
Chen, Can
Xu, Ying
Yu, Jingdi
Qian, Shenxian
Xie, Yaping
A first case of successful using of ibrutinib in treating paraneoplastic pemphigus related bronchiolitis obliterans concurrent with CLL
title A first case of successful using of ibrutinib in treating paraneoplastic pemphigus related bronchiolitis obliterans concurrent with CLL
title_full A first case of successful using of ibrutinib in treating paraneoplastic pemphigus related bronchiolitis obliterans concurrent with CLL
title_fullStr A first case of successful using of ibrutinib in treating paraneoplastic pemphigus related bronchiolitis obliterans concurrent with CLL
title_full_unstemmed A first case of successful using of ibrutinib in treating paraneoplastic pemphigus related bronchiolitis obliterans concurrent with CLL
title_short A first case of successful using of ibrutinib in treating paraneoplastic pemphigus related bronchiolitis obliterans concurrent with CLL
title_sort first case of successful using of ibrutinib in treating paraneoplastic pemphigus related bronchiolitis obliterans concurrent with cll
topic Medicine
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10056216/
https://www.ncbi.nlm.nih.gov/pubmed/37007770
http://dx.doi.org/10.3389/fmed.2023.1132535
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