Clinical and Experimental Pediatrics

The Korean Pediatric Society (대한소아청소년과학회)
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Recent advances in histiocytic disorders

조직구증식증후군의 최신지견

  • Seo, Jong Jin (Division of Hematology/Oncology/BMT, Department of Pediatrics, University of Ulsan College of Medicine and Asan Medical Center)
  • 서종진 (울산대학교 의과대학 서울아산병원 소아과 종양혈액골수이식분과)
  • Received : 2007.05.15
  • Accepted : 2007.05.08
  • Published : 2007.06.15
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Abstract

The recent advances in the basic hematology and immunology have significantly enhanced the understanding of histiocytic disorders. The Histiocyte Society which was established in 1985 enabled the randomized trials for these diseases, and important knowledge regarding pathogenesis, clinical presentation, diagnosis, therapy and late consequences has been obtained. The treatment of Langerhans cell histiocytosis (LCH) has varied greatly over last decades, and is still controversial. Therapy can be reduced for low risk patients, and it is possible to discriminate early the non-responding patients with risk disease who might require more intensified treatment. Current therapy of LCH recommended by the Histiocyte Society (LCH-III protocol) is activated in 2001. Hemophaocytic histiocytosis (HLH) is fatal if diagnosis is delayed and appropriate therapy is not instituted rapidly. The diagnostic criteria for HLH is revised by the Histiocyte Society for the current treatment protocol (HLH-2004) which consists of dexamethasone, etoposide, and cyclosporin in combination with intathecal methotrexate. Hematopoietic stem cell transplantation is usually necessary for the primary HLH and recurrent secondary HLH.

Keywords

References

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