DOI: 10.12890/2025_005480
Good’s syndrome revealed by recurrent sigmoid diverticulitis
Authors
dr.attout@ch-ales.fr
Keywords
Good’s syndrome, thymoma, hypogammaglobulinaemiaAbstract
Thymus is a primary lymphoid organ whose main function is the education of the T lymphocytes. Thymoma is uncommon neoplasia derived from epithelial cells of the thymus. Patients with thymoma frequently present with autoimmune disorders, mostly myasthenia gravis. Good’s syndrome (GS) is an acquired adult-onset combined immunodeficiency typically characterised by the triad of thymoma, hypogammaglobulinaemia and increased susceptibility to recurrent infections. Immunoglobulin replacement is the main therapeutic strategy. Our knowledge of thymoma-associated immunodeficiency is predominantly derived from case reports or small series. We report the case of a patient with recurrent sigmoid diverticulitis that appeared after a thymectomy, who was subsequently found to have severe hypogammaglobulinaemia.
References
- Good RA. Agammaglobulinemia: a provocative experiment of nature. Bull Univ Minn Hosp Min Med Found 1954;26:1–19.
- Kelesidis T, Yang O. Good’s Syndrome remains a mystery after 55 years: a systematic review of the scientific evidence. Clin Immunol 2010;135:347–363. doi: 10.1016/j.clim.2010.01.006
- Zaman M, Huissoon A, Buckland M, Patel S, Alachkar H, Edgar JD, et al. Clinical and laboratory features of seventy-eight UK patients with Good’s syndrome (thymoma and hypogammaglobulinaemia). Clin Exp Immunol 2019;195:132–138. doi: 10.1111/cei.13216
- Jansen A, van Deuren M, Miller J, Litzman J, de Gracia J, Sáenz-Cuesta M, et al. Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective. Clin Immunol 2016;171:12–17. doi: 10.1016/j.clim.2016.07.025
- Sipos F, Muzes G. Good’s syndrome: brief overview of an enigmatic immune deficiency. APMIS 2023;131:698–704. doi: 10.1111/apm.13351
- Malphettes M, Gérard L, Galicier L, Boutboul D, Asli B, Szalat R, et al., for the Deficit Immunitaire de l'adulte Study Group. Good syndrome: an adult-onset immunodeficiency remarkable for its high incidence of invasive infections and autoimmune complications. Clin Infect Dis 2015;61:e13–19. doi: 10.1093/cid/civ269
- Bernard C, Frih H, Pasquet F, Kerever S, Jamilloux Y, Tronc F, et al. Thymoma associated with autoimmune diseases: 85 cases and literature review. Autoimmun Rev 2016;15:82–92. doi: 10.1016/j.autrev.2015.09.005
- Kabir A, Alizadehfar R, Tsoukas CM. Good’s syndrome: time to move on from reviewing the past. Front Immunol 2022;12:12815710. doi: 10.3389/fimmu.2021.815710
