Good's Syndrome (Thymoma with Immunodeficiency) -A case report-

Good 증후군 치험 -1예 보고-

  • Ryoo, Ji-Yoon (Department of Thoracic and Cardiovascular Surgery, Ilsan Paik Hospital, College of Medicine, Inje University)
  • 류지윤 (인제대학교 의과대학 일산백병원 흉부외과학교실)
  • Published : 2006.01.01

Abstract

Good's syndrome (thymoma with immunodeficiency) is a rare cause of combined B and T cell immunodeficiency in adults. The clinical characteristics are increased susceptibility to bacterial infection and opportunistic viral and fungal infections. The most consistent immunological abnormalities are hypogammaglobulinemia and reduced or absent B cells. This syndrome should be treated by resecting the thymoma and replacing the immunoglobulin to maintain adequate IgG values. The author experienced one case of Good's Syndrome. The patient was a 64-year-old female who had a history of frequent sinopulmonary infection. Chest CT showed Pneumonia and anterior mediastinal mass and PCNB was taken and biopsy result was thymoma. She received thymectomy and replacement of immunoglobulin to control hypogammaglobulinemia.

Good증후군은 성인에서 발생하는 B세포 와 T세포 면역 결핍증의 드문 원인 질환으로 임상적인 특징은 세군 감염 빈도의 증가 및 바이러스, 진군의 기회 감염이다. 가장 일치하는 면역학적 이상은 저감마글로부린 혈증과 B세포가 감소되거나 소실되는 것이다. 이 증후군의 치료는 흉선종의 절제 및 적절한 면역글로부린 G 역가를 유지하기 위한 면역글로부린의 투여이다. 저자는 1예의 Good증후군을 경험하였는데 환자는 64세 여자로 빈번한 비강 폐 감염의 병력을 가지고 있다. 흉부전산화단층 촬영에서 폐렴과 전 종격동 종괴의 소견을 보였으며 경피적 침 생검에서 흉선종으로 진단되었다. 환자는 흉선절제술을 시행 받았으며 저감마 글로부린혈증을 치료하기 위해서 면역글로부린을 투여 받았다.

Keywords

References

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