title: Good's syndrome and IgA monoclonal gammopathy of undetermined significance.
abstract: Characterised by the association of a thymoma, hypogammaglobulinaemia, and B-cell and T-cell dysfunction, Good's syndrome (GS) is a rare cause of adult immunodeficiency leading to recurrent infections, and autoimmune manifestations related to the thymoma. We describe a 70-year-old woman in whom the diagnosis of GS was made after 7 years follow-up of a monoclonal gammopathy of undetermined significance (MGUS). After thymectomy, she received monthly intravenous immunoglobulin perfusions in order to maintain a normal plasmatic IgG level. To our knowledge, this is the fifth described case of GS associated with an MGUS. This rare condition should not be misdiagnosed, as the prognosis is determined by infectious and autoimmune complications, which could be prevented.
authors.affiliation: Department of Médecine Interne, CHPF, Pirae, French Polynesia.
authors.forename: Erwan
authors.initials: E
authors.lastname: Oehler
authors.forename: Laurence
authors.initials: L
authors.lastname: Heuberger
authors.forename: Frédéric
authors.initials: F
authors.lastname: Ghawche
authors.forename: Florent
authors.initials: F
authors.lastname: Valour
mesh_terms: D000361:Agammaglobulinemia / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D000368:Aged; D001402:B-Lymphocytes / Q000276:immunology*; D003937:Diagnosis, Differential; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood*; D007153:Immunologic Deficiency Syndromes / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D008998:Monoclonal Gammopathy of Undetermined Significance / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D013577:Syndrome; D013601:T-Lymphocytes / Q000276:immunology*; D013934:Thymectomy; D013945:Thymoma / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy; D013953:Thymus Neoplasms / Q000175:diagnosis* / Q000276:immunology* / Q000628:therapy
chemical_list: D007070:Immunoglobulin A