Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.

Ygal Benhamou 1 Cyrielle Assié 1 Pierre-Yves Boelle 2, 3 Marc Buffet 4 Rana Grillberger 5 Sandrine Malot 4 Alain Wynckel 6 Claire Presne 7 Gabriel Choukroun 7 Pascale Poullin 8 François Provôt 2, 9 Didier Gruson 10 Mohamed Hamidou 11 Dominique Bordessoule 12 Jacques Pourrat 13 Jean-Paul Mira 14 Veronique Le Guern 2, 15 Claire Pouteil-Noble 16 Cedric Daubin 17 Philippe Vanhille 18 Eric Rondeau 19 Jean-Bernard Palcoux 20 Christiane Mousson 21 Cecile Vigneau 22 Guy Bonmarchand 23 Bertrand Guidet 24 Lionel Galicier 25 Elie Azoulay 2, 26 Hanspeter Rottensteiner 5 Agnes Veyradier 27 Paul Coppo 4, *
Abstract : Background. Acquired thrombotic thrombocytopenic purpura is still associated with a 10-20% death rate. So far, early prognostic factors of death could not be clearly identified. To identify prognostic factors associated with 1-month death in thrombotic thrombocytopenic purpura patients with acquired severe (< 10% of normal activity) ADAMTS13 deficiency.Design and Methods. Prospective cohort of patients included between October, 2000, and August, 2010. A validation cohort of patients was set up from September, 2010 to August, 2011. 281 (analysis cohort) and 66 (validation cohort) consecutive adult thrombotic thrombocytopenic purpura patients with acquired severe ADAMTS13 deficiency. 30-day mortality after treatment initiation according to characteristics at inclusion.Results. Non-survivors (11%) were older (P=10-6) and presented more frequently arterial hypertension (P=5.10-4) and ischemic heart disease (P=0.013). Prognosis was increasingly poor with age (p=0.004). On presentation, cerebral manifestations were more frequent in non-survivors(P=0.018) and serum creatinine level was higher (P=0.008). The most significant independent variables for determining death were age, severe cerebral involvement and LDH level >=10N. A 3-level risk score for early death was defined and confirmed in the validation cohort using these variables, with higher values corresponding to increased risk of early death.Conclusions. A risk score for early death was defined in patients with thrombotic thrombocytopenic purpura and validated on an independent cohort. This score should help to stratify early treatment and intensify patients with a worse prognosis.
Type de document :
Article dans une revue
Haematologica, Ferrata Storti Foundation, 2012, 97 (8), pp.1181-6. 〈10.3324/haematol.2011.049676〉
Liste complète des métadonnées

Littérature citée [21 références]  Voir  Masquer  Télécharger

http://www.hal.inserm.fr/inserm-00700485
Contributeur : Hervé De Villemeur <>
Soumis le : mercredi 23 mai 2012 - 11:47:13
Dernière modification le : lundi 10 septembre 2018 - 15:38:57
Document(s) archivé(s) le : vendredi 24 août 2012 - 02:25:20

Fichier

haematol.2011.049676.full.pdf
Fichiers éditeurs autorisés sur une archive ouverte

Identifiants

Citation

Ygal Benhamou, Cyrielle Assié, Pierre-Yves Boelle, Marc Buffet, Rana Grillberger, et al.. Development and validation of a predictive model for death in acquired severe ADAMTS13 deficiency-associated idiopathic thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.. Haematologica, Ferrata Storti Foundation, 2012, 97 (8), pp.1181-6. 〈10.3324/haematol.2011.049676〉. 〈inserm-00700485〉

Partager

Métriques

Consultations de la notice

2416

Téléchargements de fichiers

972