ADAMTS13 and thrombotic thrombocytopenic purpura Retrospective study at the hospital of Le Mans

作者：Bovet Julien^*; Pineau Vincent Fabienne

来源：Annales de Biologie Clinique, 2014, 72(3): 321-330.

DOI：10.1684/abc.2014.0951

• thrombotic thrombocytopenic purpura
• ADAMTS13
• plasma exchange
• rituximab

Thrombotic thrombocytopenic purpura (TTP) is a rare disease with poor prognosis in the absence of treatment. The aim of the study was to compare with the literature, the clinical, biological and therapeutic management of PTT at the hospital of Le Mans (CHM) and to identify sub groups. The criteria for inclusion were all patients hospitalized between 2006 and 2012 at CHM with a number of platelets, level of hemoglobin and ADAMTS13 activity respectively less than 150 G/L, 110 g/L and 10 %. Eleven patients were included, confirming the epidemiological level rarity of this disease and the prevalence in young women, with immune dysfunction, and Afro-Caribbean origin. Consistent with literature, patients had heterogeneous symptoms but mainly neurological symptoms. Biological results were heteregenous and also different from the diagnostic described by Amorosi. Concerning therapy, all patients underwent plasma exchange with fresh frozen plasma substitution, confirming this as a reference treatment of first intention. Sub-group analysis have highlighted three predictive factors of mortality which were: an age over 70 years of age, a renal failure and a haptoglobin level superior to 0.10 g/dL. This study reminded the clinical, biological and therapeutic recommendations about TIPP and described predictive factors of mortality.

• 出版日期2014-6