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Mediastinal tumours
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Thymoma is most often an incidental finding on a chest radiograph (28,32). Symptoms occur late and are due to mass effect or local invasions and include chest pain, cough, or dyspnoea (33). In addition, patients maybe present with parathymic syndromes (34). Approximately 30%–50% of patients with this tumour either have or will develop myasthenia gravis compared with only 10%–15% of patients with myasthenia gravis who develop thymoma (35,36). Myasthenia gravis associated with thymoma occurs most frequently in women. Hypogammaglobinaemia and pure red blood cell aplasia are present in 10% and 5% of patients with a thymoma, respectively (26). Good syndrome is diagnosed in patients with a thymoma and combined B-cell/T-cell immunodeficiency (37). Thymoma is also associated with various other autoimmune disorders, such as systemic lupus erythematosus, polymyositis, and myocarditis (7,26,38). Up to 20% of patients with thymoma have a malignant neoplasm such as lymphoma, lung cancer, or thyroid cancer.
Benign Tumors of the Lung
Published in Philip T. Cagle, Timothy C. Allen, Mary Beth Beasley, Diagnostic Pulmonary Pathology, 2008
Alberto M. Marchevsky, Ruta Gupta
Patients with intrapulmonary thymomas rarely exhibit findings of myasthenia gravis and usually develop nonspecific findings such as cough, weight loss, chest pain, fever, and/or dyspnea or are discovered incidentally on imaging studies of the chest (158–166). Ryman described an unusual patient with primary pulmonary thymoma and Good syndrome (158).
Granulocyte Colony Stimulating Factor-induced Immune Recovery Uveitis Associated with Cytomegalovirus Retinitis in the Setting of Good Syndrome
Published in Ocular Immunology and Inflammation, 2022
Cheng-Chun Tai, Yu-Jang Chao, De-Kuang Hwang
Good syndrome is a rare immunodeficiency disorder characterized by the manifestation of both thymoma and hypogammaglobinemia.3 Patients usually have low or absent B lymphocytes and a defect in T lymphocyte maturity.4 Patients with Good syndrome are susceptible to infections of encapsulated bacteria, viruses and fungi, and CMV accounts for 24% of the viral infections in such patients.4 The treatment of Good syndrome includes excision of the thymoma to prevent further metastasis and this is related to the prognosis.5 However, resection of the thymoma itself cannot restore immune status in patients with Good syndrome, and intravenous immunoglobulin may be beneficial in these patients to restore immunity.4 Treatment associated with restoring immunity should be used cautiously in patients with immunodeficiency. Although IRU is common in patients with HIV infection after taking antiretroviral agents,6 IRU has also been reported in patients with immunodeficiency but without HIV infection.7
Atypical VZV Retinitis in a Patient with Good Syndrome
Published in Ocular Immunology and Inflammation, 2018
Takenori Inomata, Miki Honda, Akira Murakami
Good syndrome, characterized by thymoma with hypogammaglobulinemia, was first reported by Dr Robert Good in 1954.1 It occurs in patients with opportunistic infections as a result of deficiency of both humoral and cell-mediated immunities. In clinical practice, Good syndrome has often been diagnosed by recurring incidences of respiratory infections, bacterial diarrhea, and skin infections.2−4 Previously, a few case reports have described cytomegalovirus (CMV) retinitis with Good syndrome;3,5,6 however, there are no reports delineating the relationship between varicella zoster virus (VZV) retinitis and Good syndrome.
Cytomegalovirus Retinitis as the First Manifestation of Good Syndrome
Published in Ocular Immunology and Inflammation, 2018
Seung Woo Lee, Yong Woo Lee, Jeong Hun Bae
Good syndrome is a very rare condition comprising thymoma and adult-onset immunodeficiency and develops in 3–6% of patients with thymoma.8,9 Immunodeficiency in Good syndrome is characterized by hypogammaglobulinemia, a low peripheral B cell count, and, occasionally, defects in immunity with CD4+ T cells and an inverted CD4/CD8+ T cell ratio. However, the pathogenesis of immunodeficiency in Good syndrome is not clearly understood.