CAJ | 학술논문

介绍第56届美国血液学会(ASH)年会有关铁过载治疗的新靶点.铁沉积是β-地中海贫血和遗传性血色病的主要致死原因.这两种疾病的铁调素水平下调促进了铁的吸收,导致很多器官出现铁蓄积毒性.有研究指出,阻止铁吸收有助于减轻或阻止这两种疾病的铁过载.新的研究方向是Tmprss6,其主要抑制铁调素的表达.其他治疗方向包括减轻-地中海贫血的无效红细胞生成和贫血,因为红细胞生成旺盛抑制铁调素表达.文章主要讨论了控制铁代谢和红细胞生成的关键因素,探讨减轻或阻止这两种疾病的铁过载和减轻β-地中海贫血的贫血症状的新方法.
개소제56계미국혈액학회(ASH)년회유관철과재치료적신파점.철침적시β-지중해빈혈화유전성혈색병적주요치사원인.저량충질병적철조소수평하조촉진료철적흡수,도치흔다기관출현철축적독성.유연구지출,조지철흡수유조우감경혹조지저량충질병적철과재.신적연구방향시Tmprss6,기주요억제철조소적표체.기타치료방향포괄감경-지중해빈혈적무효홍세포생성화빈혈,인위홍세포생성왕성억제철조소표체.문장주요토론료공제철대사화홍세포생성적관건인소,탐토감경혹조지저량충질병적철과재화감경β-지중해빈혈적빈혈증상적신방법.
New progress of guidelines for novel targets to iron overload in the 56th American Society of Hematology (ASH) annual meeting was reviewed.β-thalassemia and hereditary hemochromatosis disorders,and inappropriately low levels of the liver hormone hepcidin are responsible for the increased iron absorption,leading to toxic iron accumulation in many organs.Several studies have shown that targeting iron absorption could be beneficial to reducing or preventing iron overload in these 2 disorders.New approaches target Tmprss6.Additional strategies in β-thalassemia are showing beneficial effects in ameliorating ineffective erythropoiesis and anemia.The goal of this review is to discuss the major factors controlling iron metabolism and erythropoiesis and to discuss potential novel therapeutic approaches to reduce or prevent iron overload in these 2 disorders and ameliorate anemia in β-thalassemia.