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
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
1秒前
wwj发布了新的文献求助100
1秒前
4秒前
Orange应助蓝毗尼采纳,获得10
4秒前
4秒前
4秒前
深情安青应助yang采纳,获得30
4秒前
小苔藓完成签到,获得积分10
4秒前
fugu0完成签到,获得积分10
5秒前
5秒前
君君发布了新的文献求助10
5秒前
情怀应助nanmu采纳,获得10
5秒前
爆米花应助蝴蝶能飞多远采纳,获得10
6秒前
6秒前
疯狂的善愁完成签到,获得积分20
6秒前
肖肖发布了新的文献求助10
7秒前
Sunny发布了新的文献求助10
7秒前
小蘑菇应助hhh采纳,获得10
7秒前
7秒前
7秒前
Albert发布了新的文献求助30
7秒前
AA发布了新的文献求助10
8秒前
9秒前
kofd完成签到,获得积分10
10秒前
10秒前
Chenzhs完成签到,获得积分10
10秒前
SYLH应助XingHaiZHF采纳,获得10
10秒前
xiaolin发布了新的文献求助10
10秒前
DDT完成签到,获得积分10
11秒前
11秒前
打打应助specialling采纳,获得10
12秒前
打打应助无私的谷槐采纳,获得10
13秒前
领导范儿应助科研通管家采纳,获得10
13秒前
顾矜应助科研通管家采纳,获得10
13秒前
Hello应助科研通管家采纳,获得10
13秒前
cdercder应助科研通管家采纳,获得20
13秒前
所所应助科研通管家采纳,获得10
13秒前
13秒前
13秒前
13秒前
高分求助中
Production Logging: Theoretical and Interpretive Elements 2700
Mechanistic Modeling of Gas-Liquid Two-Phase Flow in Pipes 2500
Kelsen’s Legacy: Legal Normativity, International Law and Democracy 1000
Handbook on Inequality and Social Capital 800
Conference Record, IAS Annual Meeting 1977 610
Interest Rate Modeling. Volume 3: Products and Risk Management 600
Interest Rate Modeling. Volume 2: Term Structure Models 600
热门求助领域 (近24小时)
化学 材料科学 生物 医学 工程类 有机化学 生物化学 物理 纳米技术 计算机科学 内科学 化学工程 复合材料 基因 遗传学 物理化学 催化作用 量子力学 光电子学 冶金
热门帖子
关注 科研通微信公众号,转发送积分 3546536
求助须知:如何正确求助?哪些是违规求助? 3123667
关于积分的说明 9356348
捐赠科研通 2822331
什么是DOI,文献DOI怎么找? 1551314
邀请新用户注册赠送积分活动 723326
科研通“疑难数据库(出版商)”最低求助积分说明 713699