Shinsaku IMASHUKU
Rheumatology Quarterly - 2026;4(3):231-234
Good's syndrome (GS) is a thymoma-related immunodeficiency disorder. A 54-year-old female developed a thymoma (histology; B1), which was totally resected, and post-operative irradiation (50Gy/25 fractions) was given. Five years after the thymectomy, she developed her first pneumonia, and during the successive 6 years, she suffered from bronchial asthma and frequent sinusitis. During this time, the patient was not tested for serum immunoglobulin (Ig) levels. At the age of 65 years, she was noted to have extremely low Igs (IgG 354 mg/dL, IgA 29 mg/dL, IgM <5 mg/dL, and IgE <1 IU/mL). She was also found to be deficient in B cells. Under the diagnosis of GS, monthly intravenous immunoglobulin (IVIG) was started. For 3 years, the patient remained clinically stable, until thrombocytopenia followed by refractory chronic diarrhea developed. In the management of GS, IVIG is essential to keep the patient's quality of life but may not prevent the development of other immune-mediated complications.