Thymoma with immunodeficiency
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| Thymoma with immunodeficiency | |
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| Other names | Good syndrome |
Thymoma with immunodeficiency (also known as "Good syndrome") is a rare disorder that occurs in adults in whom hypogammaglobulinemia, deficient cell-mediated immunity, and thymoma (usually benign) may develop almost simultaneously.[1]: 82 [2] Most reported cases are in Europe, though it occurs globally.[3]
Dr. Robert Good was first to describe the association between thymoma and hypogammaglobulinemia in 1954.[4] Much remains to be understood about its pathogenesis.[5]
Signs and symptoms
[edit]Most patients present with an immunodeficient state and recurrent sinopulmonary infections in their 4th or 5th decade of life. The immunodeficiency may occur before or after the diagnosis of a thymoma.[4]
Immunodeficiency involves both deficient humoral and cellular immunity. Patients have low total serum antibodies. The thymoma may inhibit the thymus’s normal role in production of self-tolerant T lymphocytes. These T-lymphocytes then attack the B cell precursors in the marrow, preventing maturation and ultimately resulting in hypogammaglobulinemia.
It is characterized by increased susceptibility to bacterial, viral, and fungal infections.[6] Good Syndrome is associated with other autoimmune conditions including pure red cell aplasia[7] and myasthenia gravis.[4]
Pathogenesis
[edit]The cause of Good Syndrome is unknown. It is thought to be an autoimmune process affecting the bone marrow.[3]
Diagnosis
[edit]Definition
[edit]There are no formal diagnostic criteria.[5] Generally it can be defined as an adult-onset primary immunodeficiency associated with thymoma, hypogammaglobulinemia, diminished B and T cells, and inverted CD4/CD8+ ratio.[2] It has been suggested that Good Syndrome is a subset of common variable immunodeficiency (CVID).[3]
Treatment
[edit]The mainstay of treatment consists of thymectomy and immunoglobulin replacement with intravenous immunoglobulin. Immunodeficiency does not resolve after thymectomy. Immunosuppression is sometimes used.[2]
The Centers for Disease Control and Prevention recommend pneumococcal, meningococcal, and Hib vaccination in those with diminished humoral and cell-mediated immunity.
Some have advocated prophylaxis with trimethoprim-sulfamethoxazole if CD4 counts are lower than 200 cells/mm^3, similar to HIV/AIDS patients.
See also
[edit]References
[edit]- ^ James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: Clinical Dermatology. Saunders Elsevier. ISBN 0-7216-2921-0.
- ^ a b c Miyakis, Spiros; Pefanis, Angelos; et al. (2006). "Thymoma with immunodeficiency (Good's syndrome): Review of the literature apropos three cases". Scandinavian Journal of Infectious Diseases. 38 (4): 314–320. doi:10.1080/00365540500372663. PMID 16718939. S2CID 44472631.
- ^ a b c Kelesidis, Theodoros; Otto, Yang (2010). "Good's syndrome remains a mystery after 55 years: A systematic review of the scientific evidence". Clinical Immunology. 135 (3): 347–363. doi:10.1016/j.clim.2010.01.006. PMC 8071094. PMID 20149753.
- ^ a b c Kelleher, P; Misbah, S A (2003). "What is Good's syndrome? Immunological abnormalities in patients with thymoma". Journal of Clinical Pathology. 56 (1): 12–16. doi:10.1136/jcp.56.1.12. PMC 1769851. PMID 12499426.
- ^ a b Jansen, Anne; van Deuren, Marcel; et al. (2016). "Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective". Clinical Immunology. 171: 12–17. doi:10.1016/j.clim.2016.07.025. hdl:2066/171270. PMID 27497628.
- ^ Grammatikos, A., Bright, P., Pearson, J. et al. Chronic Enteroviral Meningoencephalitis in a Patient with Good’s Syndrome Treated with Pocapavir. J Clin Immunol (2022). https://doi.org/10.1007/s10875-022-01321-6
- ^ Hirokawa, Makoto; Sawada, Ken-ichi; et al. (January 2008). "Long-term response and outcome following immunosuppressive therapy in thymoma-associated pure red cell aplasia: a nationwide cohort study in Japan by the PRCA collaborative study group". Haematologica. 93 (1): 27–33. doi:10.3324/haematol.11655. PMID 18166782.