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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]
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]
The cause of Good Syndrome is unknown. It is thought to be an autoimmune process affecting the bone marrow. [3]
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]
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.