Hematopoietic Reconstitution in a Patient with Fanconi's Anemia by Means of Umbilical-Cord Blood from an HLA-Identical Sibling

医学 脐带 造血 范科尼贫血 兄弟姐妹 救世主兄弟 人类白细胞抗原 贫血 脐带血 免疫学 遗传学 内科学 干细胞 疾病 抗原 DNA 生物 造血干细胞移植 DNA修复 发展心理学 心理学
作者
Éliane Gluckman,Hal E. Broxmeyer,Arleen D. Auerbach,Henry S. Friedman,Gordon C. C. Douglas,A Devergié,Hélène Espérou,Dominique Thierry,Gerard Socié,Pierre Lehn,Scott Cooper,Denis English,Joanne Kurtzberg,Judith Bard,Edward A. Boyse
出处
期刊:The New England Journal of Medicine [New England Journal of Medicine]
卷期号:321 (17): 1174-1178 被引量:1972
标识
DOI:10.1056/nejm198910263211707
摘要

The clinical manifestations of Fanconi’s anemia, an autosomal recessive disorder, include progressive pancytopenia, a predisposition to neoplasia, and nonhematopoietic developmental anomalies [1-3]. Hypersensitivity to the clastogenic effect of DNA-cross-linking agents such as diepoxybutane acts as a diagnostic indicator of the genotype of Fanconi’s anemia, both prenatally and postnatally [3-6]. Prenatal HLA typing has made it possible to ascertain whether a fetus is HLA-identical to an affected sibling [7]. We report here on hematopoietic reconstitution in a boy with severe Fanconi’s anemia who received cryo-preserved umbilical-cord blood from a sister shown by prenatal testing to be unaffected by the disorder, to have a normal karyotype, and to be HLA-identical to the patient. We used a pretransplantation conditioning procedure developed specifically for the treatment of such patients [8]; this technique makes use of the hypersensitivity of the abnormal cells to alkylating agents that cross-link DNA [9,10] and to irradiation [11] In this case, the availability of cord blood obviated the need for obtaining bone marrow from the infant sibling. This use of cord blood followed the suggestion of one of us that blood retrieved from umbilical cord at delivery, usually discarded, might restore hematopoiesis – a proposal supported by preparatory studies by some of us [12] and consistent with reports on the presence of hematopoietic stem and multipotential (CFU-GEMM), erythroid (BFU-E), and granulocyte-macrophage (CFU-GM) progenitor cells in human umbilical-cord blood (see the references cited by Broxmeyer et al. [12]).
最长约 10秒,即可获得该文献文件

科研通智能强力驱动
Strongly Powered by AbleSci AI
更新
大幅提高文件上传限制,最高150M (2024-4-1)

科研通是完全免费的文献互助平台,具备全网最快的应助速度,最高的求助完成率。 对每一个文献求助,科研通都将尽心尽力,给求助人一个满意的交代。
实时播报
刚刚
Orange应助光亮的初曼采纳,获得10
1秒前
西兰花的科研小助手完成签到,获得积分10
1秒前
lull发布了新的文献求助10
2秒前
科研通AI2S应助左丘冥采纳,获得10
3秒前
3秒前
首席医官完成签到,获得积分10
5秒前
cccxy完成签到,获得积分10
8秒前
8秒前
在水一方应助bangyang采纳,获得10
8秒前
wxzk发布了新的文献求助10
8秒前
奋斗含巧完成签到,获得积分10
9秒前
dundundun完成签到,获得积分20
9秒前
猫咪老师应助hjy采纳,获得30
10秒前
12秒前
zby关注了科研通微信公众号
13秒前
13秒前
dundundun发布了新的文献求助10
13秒前
科研通AI2S应助jjb123666采纳,获得10
15秒前
Royce关注了科研通微信公众号
15秒前
李某某完成签到,获得积分10
16秒前
nczpf2010发布了新的文献求助10
16秒前
19秒前
19秒前
甜蜜的泥猴桃关注了科研通微信公众号
20秒前
何楠楠完成签到,获得积分10
21秒前
myff完成签到 ,获得积分10
22秒前
25秒前
聪明的肉肉完成签到,获得积分20
25秒前
25秒前
邵翎365完成签到,获得积分10
27秒前
英俊的铭应助nczpf2010采纳,获得10
27秒前
尾巴藏好了完成签到,获得积分10
27秒前
cccxy发布了新的文献求助10
29秒前
zby发布了新的文献求助10
30秒前
明亮师完成签到 ,获得积分10
30秒前
安详大门完成签到,获得积分10
30秒前
研友_VZG7GZ应助高会和采纳,获得10
32秒前
Soso关注了科研通微信公众号
33秒前
传奇3应助甜甜丑采纳,获得10
34秒前
高分求助中
歯科矯正学 第7版(或第5版) 1004
SIS-ISO/IEC TS 27100:2024 Information technology — Cybersecurity — Overview and concepts (ISO/IEC TS 27100:2020, IDT)(Swedish Standard) 1000
Smart but Scattered: The Revolutionary Executive Skills Approach to Helping Kids Reach Their Potential (第二版) 1000
Semiconductor Process Reliability in Practice 720
GROUP-THEORY AND POLARIZATION ALGEBRA 500
Mesopotamian divination texts : conversing with the gods : sources from the first millennium BCE 500
Days of Transition. The Parsi Death Rituals(2011) 500
热门求助领域 (近24小时)
化学 医学 生物 材料科学 工程类 有机化学 生物化学 物理 内科学 纳米技术 计算机科学 化学工程 复合材料 基因 遗传学 催化作用 物理化学 免疫学 量子力学 细胞生物学
热门帖子
关注 科研通微信公众号,转发送积分 3233254
求助须知:如何正确求助?哪些是违规求助? 2879834
关于积分的说明 8212896
捐赠科研通 2547289
什么是DOI,文献DOI怎么找? 1376718
科研通“疑难数据库(出版商)”最低求助积分说明 647683
邀请新用户注册赠送积分活动 623115