A Case of Hemophagocytic Lymphohistiocytosis in a Child with Systemic Lupus Erythematosus

전신성 홍반성 루푸스 환아에서 병발한 혈구 탐식성 조직구 증식증 1례

  • Hwang, Ja Young (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • No, Suk Man (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Lee, Jin (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Jang, Pil Sang (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Kim, Young Hoon (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Kim, Jin Tack (Department of Pediatrics, College of Medicine, The Catholic University of Korea) ;
  • Lee, Joon Sung (Department of Pediatrics, College of Medicine, The Catholic University of Korea)
  • 황자영 (가톨릭대학교 의과대학 소아과학교실) ;
  • 노석만 (가톨릭대학교 의과대학 소아과학교실) ;
  • 이진 (가톨릭대학교 의과대학 소아과학교실) ;
  • 장필상 (가톨릭대학교 의과대학 소아과학교실) ;
  • 김영훈 (가톨릭대학교 의과대학 소아과학교실) ;
  • 김진택 (가톨릭대학교 의과대학 소아과학교실) ;
  • 이준성 (가톨릭대학교 의과대학 소아과학교실)
  • Received : 2003.04.28
  • Accepted : 2003.07.11
  • Published : 2003.10.15

Abstract

Hemophagocytic lymphohistiocytosis is a reactive disorder characterized by a generalized non-malignant histiocytic proliferation with prominent hemophagocytosis by stimulated histiocytes in the bone marrow and reticuloendothelial systems resulting in pancytopenia and liver dysfunction. Several diseases including infection, malignancy and autoimmune disease are known to be causative disorders. This case demonstrated histiocytic hemophagocytosis in the bone marrow, resulting in pancytopenia during treatment of systemic lupus erythematosus and did not show any underlying disease.

본 저자들은 전신성 홍반성 루푸스와 미만성 증식성 사구체신염으로 진단된 남아의 치료과정 중 발열과 혈구 감소증이 지속된 환아에서 이차성 혈구 탐식성 조직구 증식증을 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

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