Thrombotic thrombocytopenic purpura: a case report

被引：0

作者：

Garcia-Hejl, C. ^{[1
]}

Fagot, T. ^{[2
]}

Foissaud, V. ^{[1
]}

Samson, T. ^{[1
]}

Defuentes, G. ^{[3
]}

Clavier, B. ^{[4
]}

Perez, J. -P. ^{[5
]}

de Revel, T. ^{[2
]}

机构：

[1] HIA Percy, Biol Lab, Clamart, France

[2] HIA Percy, Serv Hermatol Clin, Clamart, France

[3] HIA Percy, Serv Med Interne, Clamart, France

[4] HIA Percy, Ctr Transfus Sanguine Armees, Clamart, France

[5] HIA Percy, Serv Anesthesie Reanimat, Clamart, France

来源：

ANNALES DE BIOLOGIE CLINIQUE | 2008年 / 66卷 / 03期

关键词：

ADAMTS; 13; thrombotic thrombocytopenic purpura; thrombotic microangiopathy;

D O I：

10.1684/abc.2008.0206

中图分类号：

R446 [实验室诊断]; R-33 [实验医学、医学实验];

学科分类号：

1001 ;

摘要：

We report a case of thrombotic thrombocytopenic purpura (TTP) in a 60 years-old woman with Sjogren's syndrome. Symptomatology on admission leads to evoke the diagnosis of TTP. Biological results allow to set the diagnosis. Actually, association of haemolytic anaemia, schizocytes and thrombocytopenia are in favour of TTP. Undetectable ADAMTS 13 activity (below 5%) confirms the diagnosis. In congenital TTP, plasma ADAMTS 13 is absent or severely reduced as a consequence of mutations in the two ADAMTS 13 gene. In acquired TTP, circulating antibodies inhibit plasma ADAMTS 13 activity. In those cases, further biological studies are needed to find a cause of TTP. Follow-up implies standard laboratory tests. Plasma exchanges are progressively tapered after normalization of platelets count.

引用

页码：327 / 331

页数：5

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