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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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2019
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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. |
format | Online Article Text |
id | pubmed-6883564 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
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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