title: [ADAMTS13 and thrombotic thrombocytopenic purpura retrospective study at the hospital of Le Mans].
abstract: 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 TTP and described predictive factors of mortality.
authors.affiliation: Laboratoire hématologie-hémostase, Centre hospitalier du Mans, Le Mans, France.
authors.forename: Julien
authors.initials: J
authors.lastname: Bovet
authors.affiliation: Laboratoire hématologie-hémostase, Centre hospitalier du Mans, Le Mans, France.
authors.forename: Fabienne
authors.initials: F
authors.lastname: Pineau-Vincent
mesh_terms: D051722:ADAM Proteins / Q000097:blood*; D000071120:ADAMTS13 Protein; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D005006:Ethnicity; D005260:Female; D005602:France / Q000453:epidemiology; D006242:Haptoglobins / Q000032:analysis; D006454:Hemoglobins / Q000032:analysis; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange / Q000706:statistics & numerical data; D010976:Platelet Count; D015995:Prevalence; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000453:epidemiology*; D051437:Renal Insufficiency / Q000453:epidemiology; D012189:Retrospective Studies; D012737:Sex Factors; D055815:Young Adult; D014841:von Willebrand Factor / Q000032:analysis*
chemical_list: D006242:Haptoglobins; D006454:Hemoglobins; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
keywords: ADAMTS13; plasma exchange; rituximab; thrombotic thrombocytopenic purpura