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Thymoma complicated with myasthenia gravis and Good syndrome – a therapeutic conundrum: a case report

BACKGROUND: Thymomas are known to be associated with myasthenia gravis and Good syndrome. Good syndrome is the association of thymoma with combined B cell and T cell immunodeficiency. The combination of all three diseases has not been reported. We discuss the therapeutic dilemma of immunosuppression...

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Autores principales: Paranavitane, Shiran, Handagala, Sumana, De Silva, Rajiva, Chang, Thashi
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6883564/
https://www.ncbi.nlm.nih.gov/pubmed/31779680
http://dx.doi.org/10.1186/s13256-019-2289-z
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author Paranavitane, Shiran
Handagala, Sumana
De Silva, Rajiva
Chang, Thashi
author_facet Paranavitane, Shiran
Handagala, Sumana
De Silva, Rajiva
Chang, Thashi
author_sort Paranavitane, Shiran
collection PubMed
description BACKGROUND: Thymomas are known to be associated with myasthenia gravis and Good syndrome. Good syndrome is the association of thymoma with combined B cell and T cell immunodeficiency. The combination of all three diseases has not been reported. We discuss the therapeutic dilemma of immunosuppression in such a case. CASE PRESENTATION: A 27-year-old Sinhalese man was evaluated for persistent cough which was associated with pleuritic chest pain and was found to have pleural-based lesions in his left hemithorax. Further evaluation confirmed these lesions to be implants from a thymoma. He subsequently developed myasthenia gravis and impending myasthenic crisis precipitated by pneumonia. He was found to have hypogammaglobulinemia with low B cell counts, confirming a diagnosis of Good syndrome. Treatment with intravenously administered broad-spectrum antibiotics, acetylcholinesterase inhibitors, orally administered glucocorticoids, plasma exchange, and intravenous immunoglobulin led to clinical improvement. He subsequently underwent thymectomy and debulking of the tumor and was maintained on regular intravenous immunoglobulins combined with low-dose prednisolone. CONCLUSIONS: Regular intravenous immunoglobulins combined with low-dose immunosuppression in addition to thymectomy appear to be safe when myasthenia gravis occurs in association with Good syndrome.
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spelling pubmed-68835642019-12-03 Thymoma complicated with myasthenia gravis and Good syndrome – a therapeutic conundrum: a case report Paranavitane, Shiran Handagala, Sumana De Silva, Rajiva Chang, Thashi J Med Case Rep Case Report BACKGROUND: Thymomas are known to be associated with myasthenia gravis and Good syndrome. Good syndrome is the association of thymoma with combined B cell and T cell immunodeficiency. The combination of all three diseases has not been reported. We discuss the therapeutic dilemma of immunosuppression in such a case. CASE PRESENTATION: A 27-year-old Sinhalese man was evaluated for persistent cough which was associated with pleuritic chest pain and was found to have pleural-based lesions in his left hemithorax. Further evaluation confirmed these lesions to be implants from a thymoma. He subsequently developed myasthenia gravis and impending myasthenic crisis precipitated by pneumonia. He was found to have hypogammaglobulinemia with low B cell counts, confirming a diagnosis of Good syndrome. Treatment with intravenously administered broad-spectrum antibiotics, acetylcholinesterase inhibitors, orally administered glucocorticoids, plasma exchange, and intravenous immunoglobulin led to clinical improvement. He subsequently underwent thymectomy and debulking of the tumor and was maintained on regular intravenous immunoglobulins combined with low-dose prednisolone. CONCLUSIONS: Regular intravenous immunoglobulins combined with low-dose immunosuppression in addition to thymectomy appear to be safe when myasthenia gravis occurs in association with Good syndrome. BioMed Central 2019-11-29 /pmc/articles/PMC6883564/ /pubmed/31779680 http://dx.doi.org/10.1186/s13256-019-2289-z Text en © The Author(s). 2019 Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.
spellingShingle Case Report
Paranavitane, Shiran
Handagala, Sumana
De Silva, Rajiva
Chang, Thashi
Thymoma complicated with myasthenia gravis and Good syndrome – a therapeutic conundrum: a case report
title Thymoma complicated with myasthenia gravis and Good syndrome – a therapeutic conundrum: a case report
title_full Thymoma complicated with myasthenia gravis and Good syndrome – a therapeutic conundrum: a case report
title_fullStr Thymoma complicated with myasthenia gravis and Good syndrome – a therapeutic conundrum: a case report
title_full_unstemmed Thymoma complicated with myasthenia gravis and Good syndrome – a therapeutic conundrum: a case report
title_short Thymoma complicated with myasthenia gravis and Good syndrome – a therapeutic conundrum: a case report
title_sort thymoma complicated with myasthenia gravis and good syndrome – a therapeutic conundrum: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6883564/
https://www.ncbi.nlm.nih.gov/pubmed/31779680
http://dx.doi.org/10.1186/s13256-019-2289-z
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