Good syndrome D81.8

Robert Good, 1954

Good's syndrome, also called thymoma with immunodeficiency, is a form of immunodeficiency occurring in adults in association with thymoma. Good syndrome is associated with other autoimmune diseases: pure red cell aplasia, pernicious anemia, diabetes mellitus, idiopathic thrombocytopenia, myasthenia gravis, lichen planus (Tavakol M et al. 2018).

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The cause of Good's syndrome is unknown. It is thought to be an autoimmune process affecting the bone marrow.

Mean age of onset: 56 years (29-75years). Good's syndrome can also occur in children, although this is extremely rare.

Most patients present with immune deficiency and recurrent sinopulmonary infections in the 4th or 5th decade of life. Immune deficiency may occur before or after the diagnosis of thymoma. Immunodeficiency includes both deficient humoral and cellular immunity. Patients have low serum total antibodies. Thymoma may inhibit the normal role of the thymus in producing self-tolerant T lymphocytes. These T lymphocytes then attack the B cell precursors in the bone marrow, preventing maturation and eventually leading to hypogammaglobulinemia.

The most common cause of infection in this patient population is recurrent sinopulmonary infection due to encapsulated organisms. The clinical presentation is similar to that of patients with X-linked agammaglobulinemia (XLA) and common variable immunodeficiency(CVID). Patients also suffer from bacterial urinary tract and skin infections. In contrast to XLA and CVID, opportunistic infections associated with disorders of cell-mediated immunity occur frequently in Good's syndrome. In particular, cytomegalovirus (CMV) colitis and retinitis, and mucocutaneous Candida infections are distinctive features of this disorder. Opportunistic infections due to herpes simplex, human herpes virus, varicella zoster, and Pneumocystis carinii pneumonia have also been described. Infectious diarrhea has been reported in almost 50% of patients with Good's syndrome: intestinal bacteria, Giardia, CMV (Kelleher P et al 2003).

There are no formal diagnostic criteria. In this respect, the diagnosis will be made by the following constellation: primary immunodeficiency in adults associated with a thymoma, hypogammaglobulinemia, decreased B and T cells and an inverted CD4/CD8+ ratio. Good's syndrome is thought to be a subset of common variable immunodeficiency (CVID).

There are no formal diagnostic criteria. In this respect, the diagnosis will be made by the following constellation: primary immunodeficiency in adults associated with a thymoma, hypogammaglobulinemia, decreased B and T cells and an inverted CD4/CD8+ ratio. Good's syndrome is thought to be a subset of common variable immunodeficiency (CVID).

The main treatment consists of thymectomy and immunoglobulin substitution with intravenous immunoglobulin.

Pneumococcal, meningococcal and Hib vaccinations for persons with reduced humoral and cell-mediated immunity are recommended.

Prophylaxis with trimethoprim-sulfamethoxazole is recommended if the CD4 count is below 200 cells/mm3 (analogous to HIV/AIDS patients).

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7. Paranavitane S et al. (2019) Thymoma complicated with myasthenia gravis and Good syndrome - a therapeutic conundrum: a case report. J Med Case Rep 13(1):348
8. Tavakol M et al (2018) Good's syndrome-Association of the Late Onset Combined Immunodeficiency with Thymoma: Review of Literature and Case Report. Iran J Allergy Asthma Immunol17:85-93.