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A case of granulomatous lung disease in a patient with Good's syndrome.

Authors :
Lee SH
Lee SM
Yang SC
Yoo CG
Kim YW
Shim YS
Han SK
Source :
The Korean journal of internal medicine [Korean J Intern Med] 2008 Dec; Vol. 23 (4), pp. 219-22.
Publication Year :
2008

Abstract

Good's syndrome is extremely rare. This adult-onset condition is characterized by a thymoma with immunodeficiency, low B- and T-cell counts, and hypo-gammaglobulinemia. The initial clinical presentation is either a mass-lesion thymoma or a recurrent infection. Patients with Good's syndrome are very susceptible to infections; common respiratory and opportunistic infections can be life-threatening. There are no reports of granulomatous lung disease in patients with Good's syndrome, although it has been observed in patients with common variable immunodeficiency, of which Good's syndrome is a subset. We describe a 53-year-old male thymoma patient who presented with respiratory symptoms caused by granulomatous lung disease and an opportunistic infection. He died of uncontrolled fungal infection despite repeated intravenous immunoglobulin and supportive care. Clinicians should look for evidence of immunologic dysfunction in thymoma patients presenting with severe recurrent infections, especially opportunistic infections.

Details

Language :
English
ISSN :
1226-3303
Volume :
23
Issue :
4
Database :
MEDLINE
Journal :
The Korean journal of internal medicine
Publication Type :
Academic Journal
Accession number :
19119261
Full Text :
https://doi.org/10.3904/kjim.2008.23.4.219