Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymoma
Good’s syndrome, also known as thymoma with combined immunodeficiency, is rare. The immunodeficiency may precede, arise concurrently with or follow the diagnosis of thymoma. In addition to myasthenia gravis and Good’s syndrome, paraneoplastic syndromes associated with thymoma can also be manifested...
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| Format: | Article |
| Language: | English |
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Wiley
2012-04-01
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| Series: | Kaohsiung Journal of Medical Sciences |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S1607551X1100235X |
| _version_ | 1819076244428292096 |
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| author | Ling-Ping Chen Jui-Shan Tsai Wei-Ming Lai Ling-Jung Yen Ming-Sun Yu Shyh-Jer Lin |
| author_facet | Ling-Ping Chen Jui-Shan Tsai Wei-Ming Lai Ling-Jung Yen Ming-Sun Yu Shyh-Jer Lin |
| author_sort | Ling-Ping Chen |
| collection | DOAJ |
| description | Good’s syndrome, also known as thymoma with combined immunodeficiency, is rare. The immunodeficiency may precede, arise concurrently with or follow the diagnosis of thymoma. In addition to myasthenia gravis and Good’s syndrome, paraneoplastic syndromes associated with thymoma can also be manifested with hematological disorders, such as pure red cell aplasia, aplastic anemia, agranulocytosis, hemolytic anemia, pernicious anemia, and paroxysmal nocturnal hemoglobinuria. Myelodysplastic syndrome is a group of clonal hematopoietic stem cell diseases characterized by cytopenia(s), dysplasia in one or more lineages, ineffective hematopoiesis, and potential precursors of acute leukemia. One proposed pathogenesis of myelodysplasia is autoantibodies that directly reject against hematopoietic cells, but this situation is rare in thymoma. Herein, we report a thymoma patient with unique paraneoplastic syndromes who developed myelodysplasia prior to Good’s syndrome. Early and accurate diagnosis of myelodysplastic syndrome is important for disease management, especially in patients whose myelodysplastic syndrome is possibly derived from autoimmunity. For thymoma patients with recurrent infections, comprehensive immunologic studies to exclude the possibility of Good’s syndrome and prophylactic intravenous immunoglobulin infusion in suitable candidates are warranted. |
| first_indexed | 2024-12-21T18:38:13Z |
| format | Article |
| id | doaj.art-cde9eb84d6a640daaedd51ca55bd61c8 |
| institution | Directory Open Access Journal |
| issn | 1607-551X |
| language | English |
| last_indexed | 2024-12-21T18:38:13Z |
| publishDate | 2012-04-01 |
| publisher | Wiley |
| record_format | Article |
| series | Kaohsiung Journal of Medical Sciences |
| spelling | doaj.art-cde9eb84d6a640daaedd51ca55bd61c82022-12-21T18:54:05ZengWileyKaohsiung Journal of Medical Sciences1607-551X2012-04-0128423624010.1016/j.kjms.2011.10.012Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymomaLing-Ping Chen0Jui-Shan Tsai1Wei-Ming Lai2Ling-Jung Yen3Ming-Sun Yu4Shyh-Jer Lin5Division of Hematology and Oncology, Kaohsiung Veterans General Hospital, Kaohsiung, TaiwanDivision of Hematology and Oncology, Kaohsiung Veterans General Hospital, Kaohsiung, TaiwanDepartment of Pathology and Laboratory Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, TaiwanDivision of Allergy, Immunology, and Rheumatology, Department of Internal Medicine, Kaohsiung Veterans General Hospital, Kaohsiung, TaiwanDivision of Hematology and Oncology, Kaohsiung Veterans General Hospital, Kaohsiung, TaiwanDivision of Hematology and Oncology, Kaohsiung Veterans General Hospital, Kaohsiung, TaiwanGood’s syndrome, also known as thymoma with combined immunodeficiency, is rare. The immunodeficiency may precede, arise concurrently with or follow the diagnosis of thymoma. In addition to myasthenia gravis and Good’s syndrome, paraneoplastic syndromes associated with thymoma can also be manifested with hematological disorders, such as pure red cell aplasia, aplastic anemia, agranulocytosis, hemolytic anemia, pernicious anemia, and paroxysmal nocturnal hemoglobinuria. Myelodysplastic syndrome is a group of clonal hematopoietic stem cell diseases characterized by cytopenia(s), dysplasia in one or more lineages, ineffective hematopoiesis, and potential precursors of acute leukemia. One proposed pathogenesis of myelodysplasia is autoantibodies that directly reject against hematopoietic cells, but this situation is rare in thymoma. Herein, we report a thymoma patient with unique paraneoplastic syndromes who developed myelodysplasia prior to Good’s syndrome. Early and accurate diagnosis of myelodysplastic syndrome is important for disease management, especially in patients whose myelodysplastic syndrome is possibly derived from autoimmunity. For thymoma patients with recurrent infections, comprehensive immunologic studies to exclude the possibility of Good’s syndrome and prophylactic intravenous immunoglobulin infusion in suitable candidates are warranted.http://www.sciencedirect.com/science/article/pii/S1607551X1100235XGood’s syndromeImmunodeficiencyMyelodysplastic syndromeParaneoplastic syndromeThymoma |
| spellingShingle | Ling-Ping Chen Jui-Shan Tsai Wei-Ming Lai Ling-Jung Yen Ming-Sun Yu Shyh-Jer Lin Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymoma Kaohsiung Journal of Medical Sciences Good’s syndrome Immunodeficiency Myelodysplastic syndrome Paraneoplastic syndrome Thymoma |
| title | Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymoma |
| title_full | Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymoma |
| title_fullStr | Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymoma |
| title_full_unstemmed | Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymoma |
| title_short | Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymoma |
| title_sort | myelodysplasia followed by good s syndrome a unique manifestation associated with thymoma |
| topic | Good’s syndrome Immunodeficiency Myelodysplastic syndrome Paraneoplastic syndrome Thymoma |
| url | http://www.sciencedirect.com/science/article/pii/S1607551X1100235X |
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