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      Hypogammaglobulinémie acquise associée au thymome: le syndrome de Good Translated title: Acquired hypogammaglobulinemia associated with thymoma: Good syndrome

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          Abstract

          Le syndrome de Good (SG) est défini par l'association d'un thymome et d'un déficit immunitaire. Il se complique souvent d’infections bactériennes broncho-pulmonaires et rhino-sinusiennes. Cette entité ne représente que 5% des syndromes para-thymiques. Ces infections respiratoires récurrentes peuvent être à l’origine de dilatation des bronches associée au syndrome de Good. Nous rapportons l’observation d’une femme âgée de 52 ans, hospitalisée pour une pneumopathie infectieuse trainante. La tomodensitométrie thoracique a permis de mettre en évidence des dilatations des bronches associées à un thymome confirmé sur pièce opératoire. La découverte d’une hypogammaglobulinémie a permis de porter le diagnostic de syndrome de Good.

          Translated abstract

          Good syndrome (GS) is defined as the association between thymoma and immune deficiency. It is often complicated by broncho-pulmonary bacterial infections and rhinosinusitis. This disease accounts for only 5% of all parathymic syndromes. These recurrent respiratory infections can cause bronchiectasis associated with Good syndrome. We report the case of a 52-year old woman hospitalized for non resolutive infectious pneumonitis. Chest CT scan showed bronchiectasis associated with thymoma confirmed by biopsy. The discovery of hypogammaglobulinemia allowed the diagnosis of Good syndrome.

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          Most cited references9

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          Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence.

          Good syndrome (GS) is a rare association of thymoma and immunodeficiency first described more than 50 years ago. However, this syndrome still remains a mystery to clinicians. We systematically reviewed all the clinical, laboratory and immunologic findings from 152 patients with Good syndrome. The syndrome has a worldwide distribution and approximately half of the cases (47%) have been described in Europe. The diagnosis of thymoma preceded the diagnosis of hypogammaglobulinemia, infection, or diarrhea in 42% of patients whereas in 38% of patients the diagnoses were made almost simultaneously within 2 months of each other. We found significant mortality in patients with this syndrome (44.5%). Astute clinical acumen and increased awareness about the clinical and immunological profile of this syndrome may increase early recognition of this syndrome and prevent mortality. Further studies are needed to elucidate this clinical entity. Copyright 2010 Elsevier Inc. All rights reserved.
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            What is Good's syndrome? Immunological abnormalities in patients with thymoma.

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              Infections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature.

              Immunodeficiency with thymoma (Good syndrome, GS) is a rare, adult-onset condition that is characterized by thymoma, hypogammaglobulinemia, and low numbers of peripheral B cells. CD4+ T lymphopenia and an inverted CD4:CD8+ T-cell ratio may be present. Here we report 5 patients with GS and infectious complications who were seen at 3 institutions between 1983 and 1999. Three patients had recurrent sinopulmonary infections, 3 had severe cytomegalovirus (CMV) disease, and 1 had Pneumocystis carinii pneumonia. Review of the literature identified 46 other reports of infections in GS patients. The infections reported in all 51 patients included recurrent sinopulmonary infection (19 cases with documented respiratory pathogens), generally with encapsulated bacteria, most often Haemophilus influenzae (11 cases); CMV disease (5 cases); bacteremia (7 cases); oral or esophageal candidiasis (6 cases); persistent mucocutaneous candidiasis (5 cases); chronic diarrhea (5 cases with documented stool pathogens); urinary tract infections (4 cases); P. carinii pneumonia (3 cases); tuberculosis (2 cases); Kaposi sarcoma (1 case); disseminated varicella (1 case); candidemia (1 case); wound infection with Clostridium perfringens (1 case); Mycoplasma arthritis (1 case); and other infections. Patients with GS present with a spectrum of sinopulmonary infections and pathogens similar to common variable immunodeficiency (CVID). Compared with patients with CVID, opportunistic infections, including severe CMV disease, P. carinii pneumonia, and mucocutaneous candidiasis, appear to be more common in patients with GS, and patients with GS may have a worse prognosis. GS should be ruled out in patients with thymoma or CVID who develop severe, especially opportunistic, infections. Treatment with intravenous immune globulin is recommended for all patients with GS.
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                Author and article information

                Journal
                Pan Afr Med J
                Pan Afr Med J
                PAMJ
                The Pan African Medical Journal
                The African Field Epidemiology Network
                1937-8688
                22 November 2017
                2017
                : 28
                : 253
                Affiliations
                [1 ]Service de Pneumologie D, Université de Tunis El Manar, Faculté de Médecine de Tunis, Hôpital Abderrahmen Mami, Ariana, Tunisie
                [2 ]Laboratoire d’Immunologie Université de Tunis El Manar, Faculté de Médecine de Tunis, Hôpital Abderrahmen Mami, Ariana, Tunisie
                [3 ]Laboratoire d’Anatomie Pathologique, Université de Tunis El Manar, Faculté de Médecine de Tunis, Hôpital Abderrahmen Mami, Ariana, Tunisie
                Author notes
                [& ]Corresponding author: Samira Aouadi, Service de Pneumologie D, Université de Tunis El Manar, Faculté de Médecine de Tunis, Hôpital Abderrahmen Mami, Ariana, Tunisie
                Article
                PAMJ-28-253
                10.11604/pamj.2017.28.253.11352
                5989270
                9f2f927b-9a70-4af9-9078-e484094898c1
                © Samira Aouadi et al.

                The Pan African Medical Journal - ISSN 1937-8688. This is an Open Access article distributed under the terms of the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 06 December 2016
                : 08 November 2017
                Categories
                Case Report

                Medicine
                thymome,hypogammaglobulinémie,syndrome de good,thymoma,hypogammaglobulinemia,good syndrome
                Medicine
                thymome, hypogammaglobulinémie, syndrome de good, thymoma, hypogammaglobulinemia, good syndrome

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