Good Syndrome in a Young Woman: An Unusual Presentation

被引:0
|
作者
Rebelo, Sandra D. [1 ]
Ferreira, Tiago [1 ]
Pacheco, Teresa [2 ,3 ,4 ]
Silva, Susana L. [5 ,6 ,7 ]
Tornada, Ana [1 ,8 ]
机构
[1] Ctr Hosp Univ Lisboa Norte, Hosp Santa Maria, Serv Med Interna, Lisbon, Portugal
[2] Ctr Hosp Univ Lisboa Norte, Hosp Santa Maria, Serv Oncol Med, Lisbon, Portugal
[3] Univ Lisbon, Serv Oncol Med, Fac Med, Lisbon, Portugal
[4] Inst Med Mol Joao Lobo Antunes, Serv Oncol Med, Lisbon, Portugal
[5] Ctr Hosp Univ Lisboa Norte, Hosp Santa Maria, Serv Imunoalergol, Lisbon, Portugal
[6] Univ Lisbon, Serv Imunoalergol, Fac Med, Lisbon, Portugal
[7] Inst Med Mol Joao Lobo Antunes, Serv Imunoalergol, Lisbon, Portugal
[8] Univ Lisbon, Serv Med Interna, Fac Med, Lisbon, Portugal
关键词
recurrent infections; hypogammaglobulinemia; immunodeficiency; thymoma; good syndrome;
D O I
10.7759/cureus.52705
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Good Syndrome is a rare disease that comprises the presence of a thymoma, immunodeficiency, and recurrent opportunistic infections. We report the case of a young woman who was diagnosed with Good Syndrome, who had a long-term history of recurrent infections, often due to atypical agents, and who also had a previous history of immunodeficiency and a B1 thymoma invading the large vessels, lung, and pericardium (Masaoka stage IV). She underwent surgical resection of the mediastinal mass, requiring vena cava superior reconstruction due to the extent of invasion, followed by adjuvant radiotherapy and immunoglobulin G supplementation. Despite relative stability in the subsequent years, without serious infections, after three years she had a thymoma recurrence requiring a new therapeutic approach. This case highlights the importance of a thorough investigation of the underlying causes of recurrent infections, which may be the result of an immunodeficiency secondary to malignancy. In young patients, early diagnosis is crucial to avoid disease progression and to reduce mortality rates. To achieve such outcomes, a multidisciplinary team and a comprehensive therapeutic strategy are necessary.
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页数:5
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