Good's syndrome is the association of thymoma with immunodeficiency, characterized by hypogammaglobulinaemia, depleted B-cells, diminished T-cells and inversion of the CD4/CD8 ratio. The initial clinical presentation is either with a mass lesion-thymoma that is usually benign, or with recurrent infections due to immunodeficiency. Thymectomy usually favourably affects associated conditions, such as pure red cell aplasia, but does not improve hypogammaglobulinaemia, thus the patient remains dependent on intravenous immune globulin and prone to infections. Infections usually affect the respiratory and/or the gastrointestinal tract. Common respiratory, opportunistic, and eventually life-threatening infections may occur. Moreover, patients with Good's syndrome may present other haematological conditions. We report 3 cases with long follow-up, sharing some common manifestations of the syndrome, but also showing unique features. The principal features of this rare syndrome are further discussed.

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http://dx.doi.org/10.1080/00365540500372663DOI Listing

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Good's syndrome is a rare secondary immunodeficiency characterised by recurrent infection and hypogammaglobulinemia associated with thymus tumour, and symptoms of recurrent infection may persist even after thymectomy. It is characterised by increased susceptibility to bacterial, viral, and fungal infections, as well as autoimmunity. Immunoglobulin replacement therapy has been reported to decrease infection rate, hospitalization, and the rate of antibiotic use in these patients.

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Objectives: Good's syndrome (GS) is a rare secondary immunodeficiency which is characterized by hypogammaglobulinemia and thymoma. This study aims to investigate the expression and distribution of B cells in thymoma tissue, given that B cells had been found to be reduced or absent in peripheral blood or bone marrow.

Methods: This study retrospectively analyzed thymoma tissues from 5 GS patients at the First Affiliated Hospital of Wenzhou Medical University and Zhejiang Provincial People's Hospital.

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