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.

• Authors: Shradha Agarwal, Charlotte Cunningham-Rundles
• Journal: Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology (Ann Allergy Asthma Immunol) Vol. 98 Issue 2 Pg. 185-90 (Feb 2007) ISSN: 1081-1206 [Print] United States
• PMID: 17304889 (Publication Type: Case Reports, Journal Article, Review)

Chemical References

• Immunoglobulins, Intravenous

Topics

• Agammaglobulinemia (complications, immunology, therapy)
• Aged, 80 and over
• B-Lymphocytes (immunology)
• CD4-CD8 Ratio
• CD4-Positive T-Lymphocytes (immunology)
• Female
• Humans
• Immunoglobulins, Intravenous (therapeutic use)
• Immunologic Deficiency Syndromes (complications, immunology, therapy)
• Male
• Middle Aged
• Thymoma (complications, immunology)
• Thymus Neoplasms (complications, immunology)