Academic Journal
Good’s Syndrome-Association of the Late Onset Combined Immunodeficiency with Thymoma: Review of Literature and Case Report
| Title: | Good’s Syndrome-Association of the Late Onset Combined Immunodeficiency with Thymoma: Review of Literature and Case Report |
|---|---|
| Authors: | Marzieh Tavakol, Seyed Alireza Mahdaviani, Mir Reza Ghaemi, Mohammad Vaezi, Atosa Dorudinia, Hamidreza Jamaati, Ali Akbar Velayati |
| Source: | Iranian Journal of Allergy, Asthma and Immunology, Vol 17, Iss 1 (2018) |
| Publisher Information: | Tehran University of Medical Sciences, 2018. |
| Publication Year: | 2018 |
| Collection: | LCC:Medicine |
| Subject Terms: | Cytomegalovirus, Hypogammaglobulinemia, Retinitis, Thymoma, Medicine |
| More Details: | Good’s syndrome, the adult onset hypogammaglobulinemia associated with thymoma has been explained about six decades ago. It generally presents with recurrent infections and several paraneoplastic syndromes including myasthenia gravis, pure red cell aplasia, connective tissue disorders, superior vena cava, Horner’s syndrome, lichen planus and inflammatory bowel disease. Lack of B cell, dysfunction of T cell, CD4+ T cell lymphopenia, reversed CD4/CD8+ T cell ratio, autoantibodies against Th17 related cytokines have been respected as the pathogenesis of the immune dysregulation this syndrome. A 57-year-old man was admitted to our hospital with a history of thymectomy due to thymoma (Type A) 6 years ago. He developed weight loss and recurrent persistent diarrhea caused by isospora belli. His chest CT scan revealed bilateral bronchiectasis. His laboratory data showed hypogammaglobulinemia and he was treated by monthly IVIG with the diagnosis of good’s syndrome. Nevertheless he referred again with left sided loss of vision because of CMV retinitis and he also developed nail candidiasis. Good’s syndrome should be considered in every patient with a history of thymoma and recurrent infection. Immunologic evaluation of these patients including measurement of the serum level of immunoglobulin as well as B cell and T cell subgroups should be performed. Physicians must be aware and think about this entity in patients with adult onset immunodeficiency. |
| Document Type: | article |
| File Description: | electronic resource |
| Language: | English |
| ISSN: | 1735-1502 1735-5249 |
| Relation: | https://ijaai.tums.ac.ir/index.php/ijaai/article/view/1115; https://doaj.org/toc/1735-1502; https://doaj.org/toc/1735-5249 |
| Access URL: | https://doaj.org/article/de442f88fcd34944ac5da96ad7cbec6a |
| Accession Number: | edsdoj.442f88fcd34944ac5da96ad7cbec6a |
| Database: | Directory of Open Access Journals |
| ISSN: | 17351502 17355249 |
|---|---|
| Published in: | Iranian Journal of Allergy, Asthma and Immunology |
| Language: | English |