Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
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Prednisolone 투여로 효과적으로 치료된 소아의 전신형 Castleman병 1례

A Case of Systemic Castleman's Disease in a Child and Successful Treatment with Oral Prednisolone

  • 구소은 (울산대학교 의과대학 소아과학교실) ;
  • 이미정 (단국대학교 의과대학 소아과학교실) ;
  • 김정은 (건국대학교 의과대학 소아과학교실) ;
  • 허주령 (울산대학교 의과대학 병리학교실) ;
  • 김태형 (울산대학교 의과대학 소아과학교실)
  • Koo, So Eun (Department of Pediatrics, University of Ulsan College of Medicine) ;
  • Lee, Mee Jeong (Department of Pediatrics, College of Medicine, Dankook University) ;
  • Kim, Jeong Eun (Department of Pediatrics, College of Medicine, Konkuk University) ;
  • Huh, Joo Ryung (Department of Pathology, University of Ulsan College of Medicine) ;
  • Ghim, Taed (Department of Pediatrics, University of Ulsan College of Medicine)
  • 투고 : 2004.10.06
  • 심사 : 2004.12.07
  • 발행 : 2005.04.15
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초록

저자들은 지속되는 발열, 빈혈, 혈소판 감소증, 흉수, 복수 및 간비종대 등의 비특이적인 전신증상을 주소로 내원한 13세 남아를 경부 림프절 조직 검사에서 혼합형의 Castleman병으로 진단한 후 스테로이드와 면역글로불린을 투여하여 호전된 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

Castleman's disease is a rare non-neoplastic lymphoproliferative disorder of unknown etiology. It is divided into three histologic subtypes; hyaline-vascular(HV), plasma cell(PC) type and mixed type (HV-PC). It has two clinical expressions. The localized form, which presents as a slow growing mass, has a relatively benign clinical course. The multicentric form is multilocated and holds significant morbidity. The mainstay of treatment of the localized form is surgical resection. The multicentric form requires medical treatment comprising prednisolone and other immunosuppressor drugs. The disease in children seems to have a more favorable course than in adults. We report a 13-year-old boy with Castleman's disease of multicentric form who was successfully treated with prednisolone and intravenous immunoglobulin.

키워드

참고문헌

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