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A Case of Thymoma with Immunodeficiency (Good's Syndrome) with Disseminated Tuberculosis

파종성 결핵을 동반한 Good 증후군 1예

  • Jung, Hyeon (Division of Infectious Diseases, Chungnam National University Hospital) ;
  • Sohn, Kyung Mok (Division of Infectious Diseases, Chungnam National University Hospital) ;
  • Kim, Yeon-Sook (Division of Infectious Diseases, Chungnam National University Hospital) ;
  • Song, Chang Hun (Division of Infectious Diseases, Chungnam National University Hospital) ;
  • Sohn, Eun Hee (Department of Neurology, Chungnam National University Hospital)
  • 정현 (충남대학교병원 감염내과) ;
  • 손경목 (충남대학교병원 감염내과) ;
  • 김연숙 (충남대학교병원 감염내과) ;
  • 송창헌 (충남대학교병원 감염내과) ;
  • 손은희 (충남대학교병원 신경과)
  • Received : 2013.01.26
  • Accepted : 2013.04.15
  • Published : 2013.09.01

Abstract

Good's syndrome is the rare association of immunodeficiency with thymoma, characterized by hypogammaglobulinemia, depleted B-cells, diminished T-cells and inversion of the CD4/CD8 ratio. Thymectomy does not usually improve hypogammaglobulinemia; thus, the patient remains prone to infections. Infections affect primarily the sinopulmonary and gastrointestinal system. Here we present a case of a 71-year-old woman with common manifestations of Good's syndrome who succumbed to disseminated tuberculosis. Serum immunoglobulin levels should be measured for patients with thymoma and susceptibility to infection.

Good 증후군은 흉선종과 면역결핍이 동반되는 드문 질환이다. 체액성 및 세포성 면역결핍이 모두 발생하며 주로 반복적인 부비동염이나 폐렴으로 발현하지만 바이러스나 진균, 결핵에 감염되기도 한다. 의심하지 않으면 진단이 늦어지는 경우가 많고 성인에서 발현하는 다른 일차성 면역 결핍증에 비해 예후가 불량하다. 치료는 면역글로불린을 주기적으로 투여한다. 저자들은 흉선종 절제술 시행 15년 후 반복적인 폐렴과 결핵에 의한 뇌수막염, 폐결핵으로 사망한 Good 증후군 증례를 경험하여 국내외 문헌고찰과 함께 보고하는 바이다.

Keywords

References

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Cited by

  1. Opportunistic lung infections as a manifestation of Good's syndrome vol.3, pp.5, 2015, https://doi.org/10.4168/aard.2015.3.5.365