Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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A case of atypical hemolytic uremic syndrome as an early manifestation of acute lymphoblastic leukemia

비전형적 용혈성 요독 증후군으로 조기 발현한 급성 림프모구성 백혈병 1예

  • Han, Dong Kyun (Departments of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School) ;
  • Baek, Hee Jo (Departments of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School) ;
  • Cho, Young Kuk (Departments of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School) ;
  • Kim, Chan Jong (Departments of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School) ;
  • Shin, Myung Geun (Laboratory Medicine, Chonnam National University Hwasun Hospital Chonnam National University Medical School) ;
  • Kook, Hoon (Departments of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School) ;
  • Hwang, Tai Ju (Departments of Pediatrics, Chonnam National University Hwasun Hospital Chonnam National University Medical School)
  • 한동균 (전남대학교 의과대학, 화순 전남대학교병원 소아과학교실) ;
  • 백희조 (전남대학교 의과대학, 화순 전남대학교병원 소아과학교실) ;
  • 조영국 (전남대학교 의과대학, 화순 전남대학교병원 소아과학교실) ;
  • 김찬종 (전남대학교 의과대학, 화순 전남대학교병원 소아과학교실) ;
  • 신명근 (전남대학교 의과대학, 화순 전남대학교병원 진단검사의학과교실) ;
  • 국훈 (전남대학교 의과대학, 화순 전남대학교병원 소아과학교실) ;
  • 황태주 (전남대학교 의과대학, 화순 전남대학교병원 소아과학교실)
  • Received : 2009.08.03
  • Accepted : 2009.12.03
  • Published : 2010.02.15
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Abstract

Hemolytic uremic syndrome (HUS) is the most common cause of acute renal failure in children younger than 4 years and is characterized by microangiopathic hemolytic anemia, acute renal failure, and thrombocytopenia. HUS associated with diarrheal prodrome is usually caused by Shiga toxin-producing Escherichia coli O157:H7 or by Shigella dysenteriae, which generally has a better outcome. However, atypical cases show a tendency to relapse with a poorer prognosis. HUS has been reported to be associated with acute lymphoblastic leukemia (ALL) in children. The characteristics and the mechanisms underlying this condition are largely unknown. In this study, we describe the case of an 11-year-old boy in whom the diagnosis of ALL was preceded by the diagnosis of atypical HUS. Thus, patients with atypical HUS should be diagnosed for the possibility of developing ALL.

용혈성 요독 증후군은 미세혈관병성 용혈성 빈혈, 급성 신부전 및 혈소판감소증을 특징으로 하며 4세 미만 소아의 급성 신부전의 가장 흔한 원인이 된다. 설사 연관형 용혈성 요독 증후군은 설사가 전구 증상으로 동반되며, shiga-toxin을 생산하는 Escherichia coli O157:H7 또는 Shigella dysenteriae 감염이 원인으로써 비교적 예후가 좋다. 하지만, 비전형적인 경우는 재발할 수 있으며 더 불량한 예후를 보인다. 소아에서는 용혈성 요독 증후군이 선행된 급성 림프모구성 백혈병은 매우 드물게 보고되며, 이들에서의 임상적인 특징이나 기저 기전은 잘 알려져 있지 않다. 이에 저자들은 11세 남아에서 비전형적 용혈성 요독 증후군 후에 발생한 급성 림프모구성 백혈병 1예를 보고하는 바이며, 이와 같은 비전형적인 경우에는 급성 림프모구성 백혈병의 가능성을 염두해 두어야 할 것으로 사료된다.

Keywords

References

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