Good Syndrome: An Adult-Onset Immunodeficiency Remarkable for Its High Incidence of Invasive Infections and Autoimmune Complications

Abstract

GS differs notably from CVID and B(-) CVID: very late onset, no familial cases, and absence of lymphoid hyperplasia. The key observation is the very high frequency of invasive bacterial infections in GS, an issue that physicians should be aware of.

Thymic Epithelial Tumor-Associated Cytopenia: A 10-Year Observational Study in France

“…This might have reduced the mortality rate in our series (22%), which was lower than those reported in various literature series (30% after 5 years in a large series published in 1993, 18 46% in a recent literature review, 16 and 30% in a very recent study 19 ). In our series, however, the four patients with GS who died (of severe lung infections) were receiving immunoglobulins but not prophylactic antibiotic therapy.…”

Features and outcomes of immunoglobulin therapy in patients with Good syndrome at Thailand’s largest tertiary referral hospital

“…Associated autoimmune diseases, including pure red cell aplasia (PRCA), myasthenia gravis (MG), oral lichen planus, aplastic anemia and primary sclerosing cholangitis had been reported. [4][5][6] Regular administration of intravenous immunoglobulin (IVIg) is the mainstay of therapy to prevent infections but cannot restore immunological abnormalities. Moreover, 5-and 10-year survival rates of GS patients were reported to be 75% and 33%, respectively which found to be lower compared with patients with common variable immunodeficiency (CVID).…”

Thymoma‐associated multi‐organ autoimmunity: A case of graft‐versus‐host disease‐like erythroderma complicated by Good syndrome successfully treated by thymectomy

“…Good syndrome is a disease entity of hypogammaglobulinemia complicated by thymoma. Patients have depleted peripheral B cells and abnormal CD4 + /CD8 + T‐cell ratio . Hypogammaglobulinemia is usually not improved by thymectomy, and some patients require prolonged Ig supplements …”