Immunodeficiency with thymoma

Good syndrome occurs in individuals all over the world, although approximately half of the cases described in the literature have been in Europe.^[1] An article published in 2010 stated that as of that year, there were less than 60 reported cases of Good syndrome worldwide.^[2] Some other published statistics related to the occurrence of Good syndrome include the following:

• Good syndrome occurs in 3-6% of individuals with thymomas.^[3]
• In the United States, the incidence of thymoma is approximately 0.15 cases per 100,000, and this tumor can be found in up to 10% of individuals with adult-onset hypogammaglobulinemia. On the other hand, the incidence of hypogammaglobulinemia is between 6–11% in individuals with thymoma.^[1]
• It has been estimated that Good syndrome is the underlying cause of immunodeficiency in 1% to 2% of individuals with primary antibody deficiency who are on immunoglobulin replacement treatments.^[1]

We are unaware of organizations that provide services or supportive resources that focus exclusively on Good syndrome; however, we have identified several groups that focus on immunodeficiencies in general. To view this information on our Web site, click here.

The long-term outlook (prognosis) for individuals with Good syndrome is believed to be worse than for those with other immunodeficiencies. The outlook is affected more by the severity of associated infections, hematologic complications, and autoimmune diseases, rather than by the behavior of the thymoma. In addition, the course of the condition may be more severe for individuals who require immunosuppressive drugs for associated autoimmune complications.

One single center review of individuals with primary antibody deficiency, spanning 20 years, reported that 70% of individuals with Good syndrome were still living 5 years after the diagnosis. At 10 years, only 33% were still living. Another review of 152 cases found significant overall mortality (46%) associated with the condition.^[1]