The Korean Journal of Medicine

  • 제81권2호
  • /
  • Pages.266-272
  • /
  • 2011
  • /
  • 1738-9364(pISSN)
  • /
  • 2289-0769(eISSN)
대한내과학회 (The Korean Association of Internal Medicine)
권호 표지

거대세포바이러스 망막염으로 발현된 Good증후군 2예

Two Cases of Cytomegalovirus Retinitis as a Manifestation of Good's Syndrome

  • 김혜인 (경북대학교 의학전문대학원 내과학교실) ;
  • 장현하 (경북대학교 의학전문대학원 내과학교실) ;
  • 박가영 (경북대학교 의학전문대학원 내과학교실) ;
  • 권유진 (경북대학교 의학전문대학원 내과학교실) ;
  • 김효훈 (경북대학교 의학전문대학원 내과학교실) ;
  • 김신우 (경북대학교 의학전문대학원 내과학교실) ;
  • 이종명 (경북대학교 의학전문대학원 내과학교실)
  • Kim, Hye-In (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Chang, Hyun-Ha (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Park, Ga-Young (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Kwon, Eu-Gene (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Kim, Hyo-Hoon (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Kim, Shin-Woo (Department of Internal Medicine, Kyungpook National University School of Medicine) ;
  • Lee, Jong-Myung (Department of Internal Medicine, Kyungpook National University School of Medicine)
  • 발행 : 2011.08.01
⟨ 이전 논문 다음 논문 ⟩

초록

Good증후군은 흉선종과 면역결핍을 나타내는 질환이다. 주로 체액성 면역결핍에 의한 세균감염이 발생하며 또한 세포성 면역결핍에 의하여 바이러스나 진균, 결핵 감염을 나타내기도 한다. Good증후군의 치료는 면역글로불린 정맥 주사이다. 시력저하를 주소로 내원한 두 환자에서 공통적으로 세포성 면역결핍에 의한 거대세포바이러스 망막염으로 발현한 Good증후군을 진단하였으며 증례 1의 환자는 폐 및 연부 조직 결핵을 동반하였고, 증례 2의 경우 거대세포바이러스 폐렴을 동반하였다. 두 환자 모두 ganciclovir 정맥주사 및 면역 글로불린 치료로 호전된 바 이에 저자들은 문헌고찰과 함께 보고하는 바이다.

Good's syndrome (GS) is a rare cause of acquired combined B- and T-cell immunodeficiency in adults. GS is also known as a rare form of paraneoplastic syndrome of thymoma that may persist after thymectomy. Some reported GS cases have been related to various diseases as a result of humoral immunodeficiency, but no report to date has related GS to cytomegalovirus (CMV) retinitis in Korea. We report two cases of CMV retinitis as a manifestation of GS with severe cellular immunodeficiency. In the first case, a 61-year-old woman was diagnosed with GS manifesting as CMV retinitis combined with coincident pulmonary tuberculosis and soft-tissue tuberculosis. In the second case, a 56-year-old man had CMV retinitis and CMV pneumonia. Both patients had a history of thymoma and had received total thymectomies, and were diagnosed with GS a few years thereafter.

키워드

참고문헌

  1. Lee SH, Lee SM, Yang SC, et al. A case of granulomatous lung disease in a patient with Good's syndrome. Korean J Intern Med 2008;23:219-222. https://doi.org/10.3904/kjim.2008.23.4.219
  2. Kelleher P, Misbah SA. What is Good's syndrome? Immunological abnormalities in patients with thymoma. J Clin Pathol 2003;56:12-16. https://doi.org/10.1136/jcp.56.1.12
  3. Tarr PE, Sneller MC, Mechanic LJ, et al. Infections in patients with Immunedeficiency with thymoma (Good's syndrome): report of 5 cases and review of the literature. Medicine (Baltimore) 2001;80:123-133. https://doi.org/10.1097/00005792-200103000-00005
  4. Yong DS, Tsang MK, Chan EY, Tse DM. Good's syndrome in a patient with cytomegalovirus retinitis. Hong Kong Med J 2008;14:142-144.
  5. Good RA. Agammaglobulinaemia a provocative experiment of nature. Bull Univ Minn Hosp Med Found 1954;26:1-19.
  6. International union of Immunological Societies. Primary immunodeficiency disease: report of an IUIS Scientific Committee. Clin Exp Immunol 1999;118:1-28.
  7. Morgenthaler TI, Brown LR, Colby TV, Harper CM Jr, Coles DT. Thymoma. Mayo Clin Proc 1993;68:1110-1123. https://doi.org/10.1016/S0025-6196(12)60907-0
  8. Song KH, Kim KH, Kim CJ, et al. A case of Good's syndrome. Infect Chemother 2007;39:259-262.
  9. Ryoo JY. Thymoma with immunodeficiency: a case report. Korean J Thorac Cardiovasc 2006;35:85-89.
  10. Kim JY, Chin JY, Lee SS, et al. A case of Good syndrome: immunodeficiency syndrome associated with a thymoma. Korean J Med 2008;75:S935-S941.
  11. Oritani K, Medina KL, Tomiyama Y, et al. Limitin: an interferonlike cytokine that preferentially influences B-lymphocyte precursors. Nat Med 2000;6:659-666. https://doi.org/10.1038/76233
  12. Charles RJ, Sabo KM, Kidd PG, Abkowitz JL. The pathophysiology of pure red cell aplasia: implications for therapy. Blood 1996;87:4831-4838.
  13. Litwin SD, Zanjani ED. Lymphocytes suppressing both immunoglobulin production and erythroid differentiation in hypogammaglobulinaemia. Nature 1977;266:57-58. https://doi.org/10.1038/266057a0
  14. Hayward AR, Paolucci P, Webster AD, Kohler P. Pre-B cell suppression by thymoma patient lymphocytes. Clin Exp Immunol 1982;48:437-442.
  15. Ide S, Koga T, Rikimaru T, Katsuki Y, Oizumi K. Good's syndrome presenting with cytomegalovirus pneumonia. Intern Med 2000;39:1094-1096. https://doi.org/10.2169/internalmedicine.39.1094