Infections in Patients with Immunodeficiency with Thymoma (Good Syndrome)

Abstract

Immunodeficiency with thymoma (Good syndrome, GS) is a rare, adult-onset condition that is characterized by thymoma, hypogammaglobulinemia, and low numbers of peripheral B cells. CD4+ T lymphopenia and an inverted CD4:CD8+ T-cell ratio may be present. Here we report 5 patients with GS and infectious complications who were seen at 3 institutions between 1983 and 1999. Three patients had recurrent sinopulmonary infections, 3 had severe cytomegalovirus (CMV) disease, and 1 had Pneumocystis carinii pneumonia. Review of the literature identified 46 other reports of infections in GS patients. The infections reported in all 51 patients included recurrent sinopulmonary infection (19 cases with documented respiratory pathogens), generally with encapsulated bacteria, most often Haemophilus influenzae (11 cases); CMV disease (5 cases); bacteremia (7 cases); oral or esophageal candidiasis (6 cases); persistent mucocutaneous candidiasis (5 cases); chronic diarrhea (5 cases with documented stool pathogens); urinary tract infections (4 cases); P. carinii pneumonia (3 cases); tuberculosis (2 cases); Kaposi sarcoma (1 case); disseminated varicella (1 case); candidemia (1 case); wound infection with Clostridium perfringens (1 case); Mycoplasma arthritis (1 case); and other infections. Patients with GS present with a spectrum of sinopulmonary infections and pathogens similar to common variable immunodeficiency (CVID). Compared with patients with CVID, opportunistic infections, including severe CMV disease, P. carinii pneumonia, and mucocutaneous candidiasis, appear to be more common in patients with GS, and patients with GS may have a worse prognosis. GS should be ruled out in patients with thymoma or CVID who develop severe, especially opportunistic, infections. Treatment with intravenous immune globulin is recommended for all patients with GS.

A Case of Good Syndrome with Pulmonary Lesions Similar to Diffuse Panbronchiolitis

“…Pure red cell aplasia and myasthenia gravis are common complications of thymoma, and improvement of these complications is often observed after thymectomy. However, Good syndrome is occasionally progressive even after thymectomy and/or corticosteroid treatment (2). In the present case, the patient's pure red cell aplasia and myasthenia gravis had been controlled after thymectomy and subsequent corticosteroid treatment, but hypoglobulinemia and recurrent lower respiratory tract infections were persistent.…”

“…While the symptoms of pure red cell aplasia and myasthenia gravis, which often occur with thymoma, are generally improved by thymectomy, Good syndrome is believed to be progressive even when thymectomy and/or corticosteroid treatment are applied (2). We herein report a case of Good syndrome that showed recurrent lower respiratory tract infections beginning nine months after a thymectomy, with chest computed tomography (CT) findings similar to diffuse panbronchiolitis (DPB).…”

Thymoma, immunodeficiency, and herpes simplex virus infections

“…The most commonly documented infectious complications in patients with thymoma with immunodeficiency include recurrent upper and lower respiratory tract infections with encapsulated organisms [1]. The patient also had chronic diarrhea, another common feature of thymoma with immunodeficiency, being reported in approximately half of the patients with this primary immunodeficiency.…”

“…Search terms included: "Good('s) syndrome", "thymoma with immunodeficiency", "thymoma", "hypogammaglobulinemia", "HSV" or "disseminated HSV". Additional cases were identified through an extended review of all the pathogens reported in 51 patients with thymoma with immunodeficiency, from 1966 to 1999 [1][2][3][4][5][6]. Reports of HSV-related infections were included if they occurred in patients with documented thymoma and hypogammaglobulinemia and/or B-cell lymphopenia [1].…”

“…Recurrent sinopulmonary infections with encapsulated bacteria are commonly reported. Patients with this primary immunodeficiency are at increased risk of developing severe opportunistic infections including herpes simplex virus (HSV)-related infections [1]. We report a case of thymoma with immunodeficiency in a patient who developed severe pneumococcal pneumonia complicated by purulent pericarditis and a fatal disseminated HSV-1 infection.…”