What is Good’s syndrome? Immunological abnormalities in patients with thymoma

• Published in: Journal of clinical pathology 2003-01, Vol.56 (1), p.12-16
• Main Authors: Kelleher, P, Misbah, S A
• Format: Article
• Language: English
• Subjects: Adult; Aged; Bacterial infections; Biological and medical sciences; Blood; Bone marrow; Chicken pox; CMV; common variable immune deficiency; computed tomography; counts per minute; cpm; CVID; Cytomegalovirus; Defects; Diarrhea; Fungal infections; Good’ syndrome; Hematology; Herpes viruses; HIV; human immunodeficiency virus; Humans; hypergammaglobulinaemia; Immunity, Cellular; Immunodeficiencies; Immunodeficiencies. Immunoglobulinopathies; immunodeficiency; Immunoglobulins; Immunologic Deficiency Syndromes - immunology; Immunological deficiency syndromes; Immunology; Immunopathology; Inflammatory bowel disease; interleukin; Lymphocytes; Medical sciences; Middle Aged; Mortality; Opportunistic Infections - immunology; Pathogenesis; PHA; phytohaemagglutinin; Pneumology; Pneumonia; Reviews; Salmonella; Streptococcus infections; Syndrome; Thymoma; Thymoma - immunology; Thymoma - surgery; Thymus Neoplasms - immunology; Thymus Neoplasms - surgery; Tumors of the respiratory system and mediastinum; Viral infections; X linked agammaglobulinaemia; XLA
• ISSN: 0021-9746; 1472-4146
• DOI: 10.1136/jcp.56.1.12

[...]patients may present with infection as a result of the defects in humoral and cellular immunity associated with a thymoma. Take home messages Good's syndrome (thymoma with immunodeficiency) is a rare cause of combined B and T cell immunodeficiency in adults The clinical characteristics of Good's syndrome are increased susceptibility to bacterial infections with encapsulated organisms and opportunistic viral and fungal infections Defects in cell mediated immunity are important causes of morbidity and mortality in this disorder and opportunistic infections such as mucocutaneous candida, cytomegalovirus infection, and Pneumocystis carinii pneumonia in patients with presumed common variable immune deficiency should alert the clinician to consider Good's syndrome The main findings on investigation of the immune system are hypogammaglobulinaemia, low or absent B cells, and variable defects in cell mediated immunity, such as CD4+ T cell lymphopenia and reduced T cell mitogen proliferative responses Treatment of Good's syndrome involves resection of the thymoma and immunoglobulin replacement to maintain adequate trough IgG values A high index of suspicion and appropriate microbiological investigations are required to diagnose and treat opportunistic infections LITERATURE SEARCH Published data for this review were identified by searching MEDLINE.