Haemophilus influenzae pneumonia and immunodeficiency in association with thymomaâA presentation of Good's Syndrome
Introduction: Good's Syndrome is a rare cause of immunodeficiency associated with thymoma. Patients with this syndrome are prone to infections with encapsulated microorganisms. The diagnosis may be delayed for a considerable time period even after the thymectomy. Case presentation: We describe...
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| Format: | Article |
| Language: | English |
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Elsevier
2011-11-01
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| Series: | Revista Portuguesa de Pneumologia |
| Online Access: | http://www.sciencedirect.com/science/article/pii/S0873215911000663 |
| _version_ | 1819097481980411904 |
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| author | A. Fahim M. Abuzakouk S.P. Hart |
| author_facet | A. Fahim M. Abuzakouk S.P. Hart |
| author_sort | A. Fahim |
| collection | DOAJ |
| description | Introduction: Good's Syndrome is a rare cause of immunodeficiency associated with thymoma. Patients with this syndrome are prone to infections with encapsulated microorganisms. The diagnosis may be delayed for a considerable time period even after the thymectomy. Case presentation: We describe the case of a 70-year-old woman with a background of thymectomy who presented with pneumonia and gram negative sepsis. Haemophilus influenzae was found in blood cultures. Moreover, there was evidence of impaired B and T cell immunity consistent with Good's Syndrome. She was commenced on immunoglobulin replacement following treatment of sepsis and remains well 18 months after the initial presentation. Conclusion: This case illustrates the importance of considering Good's Syndrome in the context of pneumonia and immunodeficiency associated with encapsulated organisms such as Haemophilus influenzae. This clinical entity is associated with a significant mortality and should be considered as a cause of immunodeficiency even years after thymectomy. Resumo: Introdução: A SÃndrome de Good é uma causa rara da imunodeficiência associada ao timoma. Os pacientes com esta sÃndrome são propensos a infecções por microrganismos encapsulados. O diagnóstico pode ser atrasado por bastante tempo, mesmo após a timectomia. Apresentação do Caso: Descrevemos o caso de uma mulher de 70 anos com antecedentes de timectomia, que apresentava pneumonia e sepsis por agente gram negativo. O Haemophilus influenzae foi isolado em hemoculturas. Além disso, evidência de alterações da imunidade celular B e T, consistente com a SÃndrome de Good. A doente iniciou terapêutica de substituição com imunoglobulina seguida de tratamento da sepsis e continua bem 18 meses após a apresentação inicial. Conclusão: Este caso ilustra a importância de considerar a SÃndrome de Good no contexto da pneumonia e imunodeficiência associadas a organismos encapsulados, como Haemophilus influenzae. Esta entidade clÃnica está associada a uma mortalidade significativa e deve ser considerada como uma causa de imunodeficiência mesmo anos depois da timectomia. Keywords: Respiratory infections, Immunodeficiency, Thymoma, Palavras-chave: Infecções respiratórias, Imunodeficiência, Timoma |
| first_indexed | 2024-12-22T00:15:47Z |
| format | Article |
| id | doaj.art-aef4081a54614f02b7dca061e78e932f |
| institution | Directory Open Access Journal |
| issn | 0873-2159 |
| language | English |
| last_indexed | 2024-12-22T00:15:47Z |
| publishDate | 2011-11-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Revista Portuguesa de Pneumologia |
| spelling | doaj.art-aef4081a54614f02b7dca061e78e932f2022-12-21T18:45:19ZengElsevierRevista Portuguesa de Pneumologia0873-21592011-11-01176272274Haemophilus influenzae pneumonia and immunodeficiency in association with thymomaâA presentation of Good's SyndromeA. Fahim0M. Abuzakouk1S.P. Hart2Division of Cardiovascular and Respiratory Studies, Castle Hill Hospital, Castle Road, Cottingham, United Kingdom; Corresponding author.Department of Immunology, Hull Royal Infirmary, Anlaby Road, Hull, United KingdomDivision of Cardiovascular and Respiratory Studies, Castle Hill Hospital, Castle Road, Cottingham, United KingdomIntroduction: Good's Syndrome is a rare cause of immunodeficiency associated with thymoma. Patients with this syndrome are prone to infections with encapsulated microorganisms. The diagnosis may be delayed for a considerable time period even after the thymectomy. Case presentation: We describe the case of a 70-year-old woman with a background of thymectomy who presented with pneumonia and gram negative sepsis. Haemophilus influenzae was found in blood cultures. Moreover, there was evidence of impaired B and T cell immunity consistent with Good's Syndrome. She was commenced on immunoglobulin replacement following treatment of sepsis and remains well 18 months after the initial presentation. Conclusion: This case illustrates the importance of considering Good's Syndrome in the context of pneumonia and immunodeficiency associated with encapsulated organisms such as Haemophilus influenzae. This clinical entity is associated with a significant mortality and should be considered as a cause of immunodeficiency even years after thymectomy. Resumo: Introdução: A SÃndrome de Good é uma causa rara da imunodeficiência associada ao timoma. Os pacientes com esta sÃndrome são propensos a infecções por microrganismos encapsulados. O diagnóstico pode ser atrasado por bastante tempo, mesmo após a timectomia. Apresentação do Caso: Descrevemos o caso de uma mulher de 70 anos com antecedentes de timectomia, que apresentava pneumonia e sepsis por agente gram negativo. O Haemophilus influenzae foi isolado em hemoculturas. Além disso, evidência de alterações da imunidade celular B e T, consistente com a SÃndrome de Good. A doente iniciou terapêutica de substituição com imunoglobulina seguida de tratamento da sepsis e continua bem 18 meses após a apresentação inicial. Conclusão: Este caso ilustra a importância de considerar a SÃndrome de Good no contexto da pneumonia e imunodeficiência associadas a organismos encapsulados, como Haemophilus influenzae. Esta entidade clÃnica está associada a uma mortalidade significativa e deve ser considerada como uma causa de imunodeficiência mesmo anos depois da timectomia. Keywords: Respiratory infections, Immunodeficiency, Thymoma, Palavras-chave: Infecções respiratórias, Imunodeficiência, Timomahttp://www.sciencedirect.com/science/article/pii/S0873215911000663 |
| spellingShingle | A. Fahim M. Abuzakouk S.P. Hart Haemophilus influenzae pneumonia and immunodeficiency in association with thymomaâA presentation of Good's Syndrome Revista Portuguesa de Pneumologia |
| title | Haemophilus influenzae pneumonia and immunodeficiency in association with thymomaâA presentation of Good's Syndrome |
| title_full | Haemophilus influenzae pneumonia and immunodeficiency in association with thymomaâA presentation of Good's Syndrome |
| title_fullStr | Haemophilus influenzae pneumonia and immunodeficiency in association with thymomaâA presentation of Good's Syndrome |
| title_full_unstemmed | Haemophilus influenzae pneumonia and immunodeficiency in association with thymomaâA presentation of Good's Syndrome |
| title_short | Haemophilus influenzae pneumonia and immunodeficiency in association with thymomaâA presentation of Good's Syndrome |
| title_sort | haemophilus influenzae pneumonia and immunodeficiency in association with thymomaaa presentation of good s syndrome |
| url | http://www.sciencedirect.com/science/article/pii/S0873215911000663 |
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