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]
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]
^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
