Thymoma-associated immunodeficiency: a good review

Provide clinicians with an overview of the latest findings while remaining pragmatic for everyday clinical practice.

More than 70 years after its description, new evidence in the etiopathogenesis of this condition is emerging, starting to create a better understanding of this disease, but most remain unknown. Recently, autoantibody against endogenous cytokines were discovered, more precisely IL-23, IL-6, IL-12, type I and II interferons. Furthermore, overall survival of Good's syndrome improved in recent times from 0% at 15 years to a 20-year survival exceeding 60%. Further studies are necessary to amalgamate current evidence incorporating all the cases described nowadays.

Good's syndrome is a rare Inborn Error of Immunity characterized by the coexistence of thymoma and immunodeficiency, the latter being defined as hypogammaglobulinemia (classical syndrome) or cellular immunity defects (nonclassical syndrome). This disease leads to predisposition to infections, autoimmunity, and malignancies, with a reduction of life expectancy. Good's syndrome management is focused on thymectomy and immunoglobulin replacement therapy. Increased disease awareness, earlier recognition, and prompt initiation of immunoglobulin replacement therapy showed an improvement in patients' survival.