Intravenous immunoglobulin replacement therapy to prevent pulmonary infection in a patient with Good\\\'s syndrome
Ching-Hsun Wang, Edward D. Chan, Cherng-Lih Perng, Chih-Feng Chian, Chien-Wen Chen, Wann-Cherng Perng, Wen-Lin Su
Received: October 5, 2016 Revised: May 2, 2012 Accepted: September 10, 2012
Corresponding author. Division of Pulmonary and Critical Care Medicine, Tri-Service General Hospital, Number 325, Section 2, Cheng-Kung Road, Neihu 114, Taipei, Taiwan.
Background and purpose:
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.
Cytomegalovirus infections, Immunodeficiency, Pneumonia, Thymoma