title: Intravenous immunoglobulin replacement therapy to prevent pulmonary infection in a patient with Good's syndrome.
abstract: Good's syndrome is an acquired immunodeficiency state associated with thymoma and characterized by recurrent pulmonary infections. We describe a 67-year-old woman who presented with respiratory symptoms caused by concomitant disseminated cytomegalovirus infection and Pneumocystis jiroveci pneumonia 38 months after thymectomy for a thymoma. Immunologic analysis revealed hypogammaglobulinemia with absent B-cell population as demonstrated by flow cytometry, consistent with Good's syndrome. Following treatment with sulfamethoxazole/trimethoprim and ganciclovir, the patient improved with resolution of her respiratory symptoms. However, the patient subsequently experienced additional infections, necessitating additional subsequent hospital admissions. During the last admission, intravenous immunoglobulin (IVIG) replacement therapy was initiated and continued after discharge. Infection has been prevented for one year after beginning IVIG replacement therapy. This case reveals that in patients with combined humoral and cell-mediated immune deficiency, concomitant infection with different pathogens is not unusual, and immediate specific therapy is important. Periodic IVIG infusion, to maintain adequate Ig levels, is recommended.
authors.affiliation: Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan.
authors.forename: Ching-Hsun
authors.initials: CH
authors.lastname: Wang
authors.affiliation: Division of Pulmonary Sciences and Critical Care Medicine, University of Colorado Denver at Anschutz Medical Center, Denver, CO 80045, USA; Denver Veterans Affairs Medical Center, Denver, CO 80220, USA; Division of Mycobacterial and Respiratory Infections, Department of Medicine, National Jewish Health, Denver, CO 80206, USA.
authors.forename: Edward D
authors.initials: ED
authors.lastname: Chan
authors.affiliation: Division of Clinical Pathology, Department of Pathology, Tri-Service General Hospital, Taipei, Taiwan; Graduate Institute of Pathology, National Defense Medical Center, Taipei, Taiwan.
authors.forename: Cherng-Lih
authors.initials: CL
authors.lastname: Perng
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan.
authors.forename: Chih-Feng
authors.initials: CF
authors.lastname: Chian
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan.
authors.forename: Chien-Wen
authors.initials: CW
authors.lastname: Chen
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan; National Defense Medical Center, Taipei, Taiwan.
authors.forename: Wann-Cherng
authors.initials: WC
authors.lastname: Perng
authors.affiliation: Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Tri-Service General Hospital, Taipei, Taiwan; National Defense Medical Center, Taipei, Taiwan. Electronic address: soa@mail.ndmctsgh.edu.tw.
authors.forename: Wen-Lin
authors.initials: WL
authors.lastname: Su
mesh_terms: D000368:Aged; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007153:Immunologic Deficiency Syndromes / Q000150:complications*; D011014:Pneumonia / Q000517:prevention & control*; D013934:Thymectomy / Q000009:adverse effects*; D013945:Thymoma / Q000150:complications* / Q000601:surgery; D016896:Treatment Outcome
chemical_list: D016756:Immunoglobulins, Intravenous