中国组织工程研究 ›› 2017, Vol. 21 ›› Issue (33): 5293-5298.doi: 10.3969/j.issn.2095-4344.2017.33.008

• 干细胞移植 stem cell transplantation • 上一篇    下一篇

MRI定量肝脏及心脏铁过载对地中海贫血全相合造血干细胞移植的影响

阳文景1,2,廖建云1,温建芸1,阮永胜1,陈丽白1,何岳林1,李春富1,吴学东1   

  1. 1南方医科大学附属南方医院儿科,广东省广州市 510515;2柳州市柳铁中心医院儿科,广西壮族自治区柳州市 545007
  • 修回日期:2017-09-12 出版日期:2017-11-28 发布日期:2017-12-01
  • 通讯作者: 吴学东,主任医师,南方医科大学南方医院儿科,广东省广州市 510515
  • 作者简介:阳文景,女,1984年生,广西壮族自治区柳州市人,汉族,南方医科大学附属南方医院在职硕士,主治医师,主要从事儿童血液学研究。现工作于柳铁中心医院。
  • 基金资助:

    广东省科技计划项目(2014A020212186);南方医院院长基金(2014B012)

Liver and cardiac iron overload is harmful to HLA-identical hematopoietic stem cell transplantation in thalassemia children: an MRI detection

Yang Wen-jing1, 2, Liao Jian-yun1, Wen Jian-yun1, Ruan Yong-sheng1, Chen Li-bai1, He Yue-lin1, Li Chun-fu1,Wu Xue-dong1   

  1. 1Department of Pediatrics, Nanfang Hospital, Southern Medical University, Guangzhou 510515, Guangdong Province, China; 2Department of Pediatrics, Liuzhou Municipal Liutie Central Hospital, Liuzhou 545007, Guangxi Zhuang Autonomous Region, China
  • Revised:2017-09-12 Online:2017-11-28 Published:2017-12-01
  • Contact: Wu Xue-dong, Chief physician, Department of Pediatrics, Nanfang Hospital, Southern Medical University, Guangzhou 510515, Guangdong Province, China
  • About author:Yang Wen-jing, Master, Attending physician, Department of Pediatrics, Nanfang Hospital, Southern Medical University, Guangzhou 510515, Guangdong Province, China; Department of Pediatrics, Liuzhou Municipal Liutie Central Hospital, Liuzhou 545007, Guangxi Zhuang Autonomous Region, China
  • Supported by:

    the Science and Technology Research Project of Guangdong Province, No. 2014A020212186; the President Fund of Nanfang Hospital, No. 2014B012

摘要:

文章快速阅读:

文题释义:
造血干细胞移植:
是指患者经化疗和/或放疗后,在造血或免疫功能极度低下的情况下,移植自体的或同种异体的造血干细胞,从而达到重建造血与免疫功能的一种新的治疗技术。自1982年第一例成功运用人类白细胞抗原全相合的同胞骨髓移植治疗重型地中海贫血以来,经过30多年的不断摸索及实践,该技术已相对成熟。成功行造血干细胞移植术的地中海贫血患儿可脱离长期输血及去铁的命运,其生活质量明显高于依赖输血及去铁的地中海贫血患儿。
铁过载:为机体含铁增加而导致组织器官功能损害的一种疾病状态,有美国学者指出:当患者输注红细胞≥ 20 U或血清铁蛋白持续> 1 000 μg/L,即可诊断铁过载。重型地中海贫血患儿需长期输血维持生命,大多数都存在铁过载。

 

摘要
背景:
大部分的重型β-地中海贫血患儿都存在铁过载的情况,而铁过载可对造血干细胞移植产生不利影响。
目的:利用MRI T2*检测肝脏及心脏铁过载情况,从而评估肝脏、心脏铁过载对重型β-地中海贫血患儿全相合异基因造血干细胞移植的影响。
方法:对81例全相合异基因造血干细胞移植、年龄>3岁,能配合MRI检测的重型β-地中海贫血患儿,进行肝脏及心脏MRI T2*检测。根据检测结果计算出肝脏及心脏铁浓度,作为评价肝心铁过载的指标,并将肝脏、心脏铁浓度与患儿血清铁蛋白、造血重建时间、死亡率、植入率、移植后常见并发症的发病率,如移植物抗宿主病、感染、免疫性溶血、全血细胞减少、肝静脉阻塞病、败血症进行相关因素分析。
结果与结论:肝脏铁浓度与血红蛋白植入时间呈正相关(r=0.229,P=0.043),心脏铁浓度与死亡率呈正相关(r=0.266,P=0.017),血清铁蛋白与植入率呈负相关(r=-0.289,P=0.009),血清铁蛋白与败血症发病率呈正相关(r=0.251,P=0.024),血清铁蛋白与全血细胞减少发病率呈正相关(r=0.276,P=0.013)。由此可见,铁过载可对重型β-地中海贫血患儿全相合异基因造血干细胞移植产生不利影响。因此,对行造血干细胞移植的β-地中海贫血患儿综合进行血清铁蛋白、肝脏及心脏铁过载的评估非常必要。

 

关键词: 干细胞, 移植, 重型β地中海贫血, 铁过载, 造血干细胞移植, 核磁共振T2*, 肝铁浓度, 心铁浓度, 血清铁蛋白, 败血症, 植入率, 血红蛋白植入时间

Abstract:

BACKGROUND: The majority of children with β-thalassemia major have iron overload, and iron overload may have negative effects on hematopoietic stem cell transplantation.
OBJECTIVE: To assess the effects of liver and cardiac iron overload detected by magnetic resonance imaging (MRI) T2* on HLA-identical allogeneic hematopoietic stem cell transplantation in children with β-thalassemia major.
METHODS: Eighty-one children with β-thalassemia major who were over 3 years of age and could cooperate with MRI detection were subjected to liver and heart MRI T2* tests before or after HLA-identical allogeneic hematopoietic stem cell transplantation. According to the test results, we calculated the liver and cardiac iron content, defined as an indicator of liver and heart iron overload. Then, there was a correlation analysis between the liver and cardiac iron content and serum ferritin, time of hematopoietic reconstitution, mortality rate, implantation rate and the morbidity of transplantation related complications, such as graft-versus-host disease, infections, autoimmune hemolysis, pancytopenia, hepatic veno-occlusive disease, septicemia.
RESULTS AND CONCLUSION: The liver iron content was positively correlated with the time of hemoglobin implantation (r=0.229, P=0.043), and the cardiac iron content were positively correlated with the mortality rate (r=0.266, P=0.017); the serum ferritin level was negatively correlated with the implantation rate (r=-0.289, P=0.009), and positively correlated with the morbidity of septicemia (r=0.251, P=0.024) and pancytopenia (r=0.276, P=0.013). Therefore, iron overload exerts negative effects on HLA-identical allogeneic hematopoietic stem cell transplantation in β-thalassemia major children, and it is necessary to detect serum ferritin level and assess liver and cardiac iron overload before cell transplantation.

 

 

Key words: beta-Thalassemia, Hematopoietic Stem Cell Transplantation, Iron Overload, Magnetic Resonance Imaging, Tissue Engineering

中图分类号: