Thymoma and immunodeficiency (Good syndrome): a report of 2 unusual cases and review of the literature

Abstract

Background: Good syndrome is a rare cause of combined B- and T-cell immunodeficiency that occurs in association with a thymoma. Patients affected with Good syndrome have increased susceptibility to bacterial, fungal, viral, and opportunistic infections.

Objective: To describe 2 unusual cases of infections in patients with Good syndrome and review the literature.

Methods: Case 1 describes a 51-year-old woman with Good syndrome who presented with a 10-day history of diarrhea, nausea, and fevers. During her hospitalization she became pancytopenic and underwent a bone marrow biopsy and evaluation of her peripheral blood smear. Case 2 describes an 89-year-old man with Good syndrome who presented with a nonhealing leg ulcer, which underwent biopsy. A literature search through MEDLINE was performed. Keywords included Good syndrome, thymoma, hypogammaglobulinemia, immunodeficiency, and infection.

Results: The peripheral blood smear in patient 1 showed ring-formed parasites in red blood cells suggestive of babesiosis. She began treatment with azithromycin, atovaquone, and doxycycline and recovered completely. Patient 2 underwent a biopsy of the foot. Immunohistochemical staining was positive for human herpesvirus 8 consistent with Kaposi sarcoma.

Conclusions: The concomitant occurrence of immunodeficiency and thymoma is known as Good syndrome. In contrast to other humoral immune defects, patients with this syndrome can develop opportunistic infections, and the prognosis appears less favorable compared with X-linked agammaglobulinemia or common variable immunodeficiency. Immunological investigations, including T-cell subsets, B cells, and quantitative immunoglobulins, should be considered part of the routine diagnostic evaluation in patients with a thymoma and recurrent infections.

Figure 1

Chest x-ray film showing right mediastinal mass. Reprinted with permission from Alan Pestronk, MD, Department of Neurology, Washington University School of Medicine, St Louis, MO.

Figure 2

Chest x-ray film showing right anterior mediastinal mass. Reprinted with permission from Alan Pestronk, MD, Department of Neurology, Washington University School of Medicine, St Louis, MO.

Figure 3

Spindle cell thymoma. Reprinted with permission of the American Society of Clinical Pathology from Moran CA, Suster S. On the histologic heterogeneity of thymic epithelial neoplasms: impact of sampling in subtyping and classification of thymomas. Am J Clin Pathol. 2000;114: 760 –766.