Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Effect of intravenous deferoxamine in multiply transfused patients

대량 수혈을 받은 환아들에서 정맥 투여한 deferoxamine의 효과

  • Oh, Sang Min (Department of Pediatrics, The Chungnam National University College of Medicine) ;
  • Kang, Joon Won (Department of Pediatrics, The Chungnam National University College of Medicine) ;
  • Kim, Sun Young (Department of Pediatrics, The Chungnam National University College of Medicine)
  • 오상민 (충남대학교 의과대학 소아과학교실) ;
  • 강준원 (충남대학교 의과대학 소아과학교실) ;
  • 김선영 (충남대학교 의과대학 소아과학교실)
  • Received : 2007.08.10
  • Accepted : 2007.10.04
  • Published : 2007.12.15
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Abstract

Purpose : Multiple transfusions in patients with chronic anemia can result in excessive iron deposition in tissues and organs. Effective iron chelation therapy in chronically transfused patients can only be achieved when iron chelators remove sufficient amounts of iron equivalent to those accumulated in the body from transfusions, thus leading to maintain body iron load at a non-toxic level. This study was retrospectively carried out to investigate the effect of intravenous iron chelation therapy with deferoxamine in patients who have received multiple transfusions. Methods : From March 2005 to January 2007, 15 patients who have received multiple transfusions were included in this study. Transfusion dependent patients were defined as those receiving >1 packed red blood cell (RBC) units/month for at least 6 months. They received intravenous deferoxamine for 7 days (10-30 mg/kg/day, 24 hour continuous infusions). Before and after deferoxamine infusions and 3 months later, we compared serum iron, TIBC, and ferritin in transfusion dependent patients and transfusion independent patients. Results : There were 6 males and 9 females and their age range was 5.6-21.3 (median 8.3) years. Transfusion dependent patients were 7 and 8 were transfusion independent states after stem cell transplantation or chemotherapy. There was no significant change in ferritin level after deferoxamine treatment for the transfusion dependent patients but significant falling of ferritin level was observed for the transfusion independent patients 3 months later compared with baseline ferritin level (P=0.046). Some adverse events were observed but symptoms were mild and tolerable. Conclusion : Seven days of intravenous deferoxamine was safe and effective in transfusion independent patients. In transfusion dependent patients, chelation therapy should be maintained, in order to minimize or prevent iron accumulation and storage in the tissues.

목 적 : 만성 빈혈로 대량 수혈을 받아야 하는 환아들에서는 조직과 장기에 철이 축적될 수 있다. 이러한 환아들에서 효과적인 철킬레이트 치료의 목적은 충분한 양의 철을 제거하여 체내에서 철로 인한 장기 손상이 나타나지 않도록 하는데 있다. 본 연구는 후향적으로 대량 수혈을 받은 환아들에서 정맥 투여한 deferoxamine의 효과를 알아보고자 하였다. 방 법 : 2005년 3월부터 2007년 1월까지 15명의 대량 수혈을 받았던 환아들을 대상으로 하였으며 이들 중 수혈 의존성 환아들은 한 달에 1단위 이상의 농축 적혈구 수혈을 최근 6개월 이상 계속 받고 있는 환아들로 정의하였다. 7일 동안 deferoxamine을 10-30 mg/kg/day로 24시간 지속 정맥주입하였으며 투여 전, 후 그리고 3개월 후의 혈청 철, 총철결합능, ferritin을 수혈 의존성 환아들과 수혈 비의존성 환아들로 나누어 비교하였다. 결 과 : 6명의 남아와 9명의 여아가 있었으며 이들의 나이는 5.6-21.3(중앙값 8.3)세였고 수혈 의존성 환아들은 7명, 수혈 비의존성 환아들은 8명이었다. 수혈 의존성 환아들의 ferritin은 deferoxamine 투여 전과 후, 3개월 후에 의미 있는 차이를 보이지 않았으나 수혈 비의존성 환아들에서는 투여 전과 비교 시 투여 3개월 후에는 의미 있는 감소를 보였다(P=0.046). Deferoxamine 정맥주입과 연관된 이상 반응은 경미하였으며 1-2일 내에 소실되었다. 결 론 : 7일 동안 정맥주입하는 deferoxamine은 수혈 비의존성 환아들에서 단기간의 효과적인 치료로 사용될 수 있을 것으로 생각되지만 수혈 의존성 환아들의 경우에는 철의 축적을 막기 위해 유지 요법이 필요할 것으로 생각된다.

Keywords

References

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