Durable hypogammaglobulinemia associated with thymoma (Good syndrome).
نویسندگان
چکیده
Good syndrome, characterized by hypogammaglobulinemia and acquired immunodeficiency, is a rare condition associated with thymoma. A 67-year-old woman, who 4 months previously had a thymoma resected, presented with generalized hypogammaglobulinemia with a severely decreased B cell population as demonstrated by flow cytometry. She was diagnosed as having bacterial mediastinitis associated with Good syndrome. For the subsequent 6 years, she suffered from repeated serious bacterial infections. As this paraneoplastic syndrome is not resolved by tumor removal, careful management with intensive infection-control using antibiotics and intravenous immunoglobulins is required for the long term. Serum immunoglobulin levels should be evaluated for patients with thymoma and suspected vulnerability to infection.
منابع مشابه
Pure Red Cell Aplasia Associated with Good Syndrome
Pure red cell aplasia (PRCA) and hypogammaglobulinemia are paraneoplastic syndromes that are rarer than myasthenia gravis in patients with thymoma. Good syndrome coexisting with PRCA is an extremely rare pathology. We report the case of a 50-year-old man with thymoma and PRCA associated with Good syndrome who achieved complete PRCA remission after thymectomy and postoperative immunosuppressive ...
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Introduction Good syndrome (thymoma with immunodeficiency) is a rare cause of immunodeficiency.1There is a combined B and T cell immunodeficiency with hypogammaglobulinemia, reversal of the CD4/CD8 ratio and reduced or absent B cells causing an increased susceptibility to bacterial, viral and fungal infections. It is classified as a primary immunodeficiency,2 and patients usually present with r...
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ورودعنوان ژورنال:
- Internal medicine
دوره 48 19 شماره
صفحات -
تاریخ انتشار 2009