作者
Eric Mariotte, Elie Azoulay, Lionel Galicier, Eric Rondeau, Fouzia Zouiti, Pierre Boisseau, Pascale Poullin, Emmanuel de Maistre, François Provôt, Yahsou Delmas, Pierre Perez, Ygal Benhamou, Alain Stepanian, Paul Coppo, Agnès Veyradier
发表日期
2016/5/1
期刊
The Lancet Haematology
卷号
3
期号
5
页码范围
e237-e245
出版商
Elsevier
简介
Background
Thrombotic thrombocytopenic purpura is a thrombotic microangiopathy related to a severe deficiency of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13; activity <10%). We aimed to investigate the association between mechanisms for ADAMTS13 deficiency and the epidemiology and pathophysiology of thrombotic thrombocytopenic purpura at initial presentation.
Methods
Between Jan 1, 1999, and Dec 31, 2013, we did a cross-sectional analysis of the French national registry for thrombotic microangiopathy to identify all patients with adult-onset thrombotic microangiopathy (first episode after age 18 years) who had severe ADAMTS13 deficiency at presentation. ADAMTS13 activity, anti-ADAMTS13 IgG, and ADAMTS13 gene mutations were investigated by a central laboratory. We collected patients' clinical data for correlation with their ADAMTS13 …
学术搜索中的文章
E Mariotte, E Azoulay, L Galicier, E Rondeau, F Zouiti… - The Lancet Haematology, 2016