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Background: Good syndrome (GS) is an adult-onset immunodeficiency characterized by coexisting thymoma and hypogammaglobulinemia. Clinical course after treatment with intravenous immunoglobulin (IVIg) has rarely been reported., Objective: To investigate and report the clinical course and outcomes of GS patients after treatment with IVIg at Thailand's largest national tertiary referral hospital METHODS: This retrospective chart review included patients diagnosed with GS and treated with IVIg during the 1 January 2005 to 31 December 2015 study period., Results: Nine GS patients with a median age at diagnosis of 53 years were included. Pneumonia and sepsis were the most common clinical manifestations. Six infectious organisms suggestive of cell-mediated immunity defect occurred in six patients, including cytomegalovirus (CMV), Mycobacterium tuberculosis, Mycobacterium abscessus, Herpes simplex virus (HSV), Pneumocystis jirovecii, and Aspergillus. Mean serum IgG level was 317 mg/dL. Eight patients had very low to undetectable B-cells. Five patients had either low CD4 number or impaired T-cell function, and one patient had both. All patients received IVIg replacement therapy monthly at a dose of 0.4 g/kg. The mean trough IgG level was 881 mg/dL. After treatment with IVIg replacement, seven patients had favorable clinical outcomes. However, two patients expired due to septicemia., Conclusion: Clinical outcomes of patients with GS are more dependent on the severity of infections and associated hematologic and autoimmune diseases than on the severity of thymoma itself. Therefore, early recognition and prompt IVIg replacement may change the natural course of this condition and may be successful in keeping the patient infections free.

This article provides a unique perspective on thymoma by describing the clinical scenarios from 2 diverse patient populations followed by an update. A comparative chart review was conducted on patients diagnosed at 2 university-based hospitals, 1 in the United States and 1 in Thailand. A comprehensive review of the literature was then performed through MEDLINE for articles between 1980 and 2002. During the last 23 years, charts from 16 patients at each institution were available for review. The most common presenting symptoms were myasthenia gravis (47%), dyspnea (25%), and chest discomfort (19%) and are similar to those reported in the literature. The primary treatment of stages I-III disease included surgery with or without radiation. Trimodality therapy with surgery, radiotherapy, and chemotherapy was pursued in 43% of patients with stage IV disease in the United States, whereas no patients from Thailand underwent this regimen. Median overall survivals were 124 and 76 months in the Thai and the US groups, respectively (P = 0.76). No major differences in the clinical features were observed between the 2 institutions, although a trend toward more advanced disease was seen in the United States. Surgery and radiation remain the backbone of treatment, but the role for chemotherapy is increasing.