Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Clinical characteristics and prognostic factors for survival in hemophagocytic lymphohistiocytosis

혈구탐식성 림프조직구증의 임상적 특징과 생존에 미치는 예후인자

  • Kim, Khi Joo (Department of Pediatrics, Yonsei University College of Medicine, Severance Children's Hospital) ;
  • Kim, Ki Hwan (Department of Pediatrics, Yonsei University College of Medicine, Severance Children's Hospital) ;
  • Lee, Taek Jin (Department of Pediatrics, Yonsei University College of Medicine, Severance Children's Hospital) ;
  • Chun, Jin-Kyong (Department of Pediatrics, Yonsei University College of Medicine, Severance Children's Hospital) ;
  • Lyu, Chuhl Joo (Department of Pediatrics, Yonsei University College of Medicine, Severance Children's Hospital) ;
  • Kim, Dong Soo (Department of Pediatrics, Yonsei University College of Medicine, Severance Children's Hospital)
  • 김기주 (연세대학교 의과대학 소아과학교실) ;
  • 김기환 (연세대학교 의과대학 소아과학교실) ;
  • 이택진 (연세대학교 의과대학 소아과학교실) ;
  • 전진경 (연세대학교 의과대학 소아과학교실) ;
  • 유철주 (연세대학교 의과대학 소아과학교실) ;
  • 김동수 (연세대학교 의과대학 소아과학교실)
  • Received : 2007.09.03
  • Accepted : 2008.10.10
  • Published : 2008.03.15
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Abstract

Purpose : Hemophagocytic lymphohistiocytosis (HLH) is a rare but fatal disorder characterized by fever, splenomegaly, pancytopenia, and hemophagocytosis in the bone marrow and other tissues. In this study, we investigated the clinical manifestations and prognostic factors in patients with HLH. Methods : We retrospectively analyzed the data from 29 patients who were diagnosed whit HLH in the Severance Children's Hospital from Jan. 1996 to Feb. 2007. Results : The median age at diagnosis was 3.8 years (range 0.1-12.2). The ratio of male to female patients was 1.1:1. The 5-year overall survival rate was 55.2% with a median follow-up duration of 32 months. In a multivariate analysis, the duration of fever before admission (survival vs. non-survival, 6.5 days vs. 14 days, P=0.010), the interval from the day of fever onset to the day of initiation of etoposide (survival vs. non-survival, 10 days vs. 35 days, P=0.002) and the presence of neurologic symptoms (survival vs. non-survival, 1 case vs. 7 cases, P=0.010) were independent, poor prognostic factors of HLH. EBV infection, gender, and the level of serum ferritin had no relations to the poor prognosis of the disease. Conclusion : This study showed that the presence of neurologic symptoms and a longer duration of fever were related to a poor prognosis. Therefore, if a patient develops neurologic symptoms and the duration of fever is prolonged, a prompt diagnostic approach and aggressive treatment for HLH are necessary.

목 적 : HLH은 발열, 범혈구감소증, 비종대, 골수에서의 혈구탐식을 특징으로 하는 질환으로 생존율이 낮고 급격한 진행양상을 보이는 비교적 드문 질환으로 생존율에 영향을 미치는 인자들을 알아보고자 본 연구를 시행하였다. 방 법 : 1996년 1월부터 2007년 2월까지 세브란스 어린이 병원에 내원하여 HLH로 진단된 환아들을 대상으로 진료기록을 토대로 후향적으로 연구를 진행하였다. 결 과 : HLH로 진단된 환아는 총 29명이었고(남아 15명, 여아 14명) 연령의 중앙값은 3.8(0.1-12.2)세였다. 가족력이 있었던 환아는 2례였다. 29명 환아의 5년 생존율은 55.2%였다. 생존군과 사망군의 통계학적으로 유의한 차이가 있었던 소견으로는 입원전 발열기간, 처음 발열일부터 etoposide를 투여시작까지의 기간 및 신경학적 이상 유무가 있었다. 입원전 발열기간은 생존환아에서는 6.5(2-21)일이었고 사망환아에서는 14(5-60)일이었으며(P=0.010) 처음 발열일부터 etoposide를 사용하기까지의 기간은 생존환아에서는 10(8-23)일, 사망환아에서는 35(15-84)일이었다(P=0.002). 또한 신경학적 이상은 생존환아에서는 1례 있었고 사망환아에서는 7례가 있었다(P=0.010). 결 론 : HLH은 발열을 주증상으로 간비비대나 범혈구 감소증, 경련이나 의식의 장애와 같은 신경학적 증상을 동반할 수 있는 질환으로 환아가 발열기간이 길거나 신경학적 증상이 발병할 경우에는 HLH의 예후가 나쁘므로 HLH 의심 시 신속한 진단적 접근과 함께 조기의 적극적인 치료가 필요할 것으로 생각된다.

Keywords

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