Case reportThymoma and immunodeficiency (Good syndrome): a report of 2 unusual cases and review of the literature
Section snippets
INTRODUCTION
Good syndrome is a rare association of thymoma and immunodeficiency first described more than 50 years ago. Patients are most commonly between the ages of 40 and 70 years and have a thymoma, low to absent B cells in the peripheral blood, hypogammaglobulinemia, and defects in cell-mediated immunity. As reviewed by Tarr et al,1 patients often present with recurrent infections due to encapsulated bacteria, fungi, and viruses. Herein, we describe 2 patients with Good syndrome and their presentation
METHODS
We searched the literature using the MEDLINE (National Library of Medicine, Bethesda, MD) database. Keywords used in the search included Good syndrome, thymoma, hypogammaglobulinemia, immunodeficiency, and infection. Additional cases were identified using references of publications found. Only articles and abstracts published in English were included.
Case 1
A 51-year-old white woman who was previously healthy presented with a persistent cough, several bouts of pneumonia, and oral ulcerations. A chest x-ray examination in May 2000 revealed a large thymoma, which was resected. Despite the resection, she continued to have oral ulcerations and a cough with shortness of breath. In October 2000, a lung biopsy specimen demonstrated fragments of alveolar parenchyma with large intra-alveolar myxoid tissue plugs consistent with cryptogenic organizing
Definition
The association between the presence of a thymoma and adult-onset hypogammaglobulinemia was first described by Dr Robert Good in 1955.2 There are a number of definitions for Good syndrome. Practice parameters in 20053 define it as a subset of common variable immunodeficiency; however, the reduced numbers of peripheral B cells noted in Good syndrome are not a feature of common variable immunodeficiency, which typically shows impaired B-cell maturation. Others choose to define it as
CONCLUSIONS
In patients with a thymoma, recognition of hypogammaglobulinemia is an important clinical consideration, since the prognosis for patients with Good syndrome appears worse when compared with common variable immunodeficiency or X-linked agammaglobulinemia.29 The major causes of death in patients with Good syndrome include infections, autoimmune diseases, and hematologic complications.1, 12 In addition, the clinical course of disease may be more severe for those patients who require
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