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Title: Features and outcomes of immunoglobulin therapy in patients with good syndrome at Thailand’s largest tertiary referral hospital
Authors: Torpong Thongngarm
Adhiratha Boonyasiri
Panitan Pradubpongsa
Nattaporn Tesavibul
Tauangtham Anekpuritanang
Piyawut Kreetapirom
Mongkhon Sompornrattanaphan
Faculty of Medicine, Siriraj Hospital, Mahidol University
Phramongkutklao College of Medicine
Chaophya Hospital
Keywords: Immunology and Microbiology;Medicine
Issue Date: 1-Jun-2019
Citation: Asian Pacific Journal of Allergy and Immunology. Vol.37, No.2 (2019), 109-115
Abstract: © 2019, Allergy and Immunology Society of Thailand. All rights reserved. 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.
ISSN: 22288694
Appears in Collections:Scopus 2019

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