title: [Update on thrombotic thrombocytopenic purpura].
abstract: OBJECTIVE
  Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder and early treatment is vital. Here, we review the recent advances in the understanding of the pathophysiology of TTP and its treatment.
  
  
  BACKGROUND
  Recent advances have shown that TTP is caused by deficiency of the (ADAMTS-13) metalloprotease that cleaves von Willebrand factor multimers. Acquired TTP is associated to inhibitory antibodies directed against ADAMTS-13. This has led to assess new therapeutic approaches in refractory and relapsing forms of TTP and the use of rituximab has shown very encouraging results.
  
  
  CONCLUSIONS
  A better characterization of TTP amongst the other thrombotic microangiopathies has allowed the use of new therapeutic approaches with the use of rituximab. The encouraging results reported with rituximab in some forms of TTP challenge the classic treatment based on plasma exchanges.
authors.affiliation: Service de médecine interne, CHU La-Conception, AP-HM, Marseille cedex 5, France. nicolas.scleinitz@ap-hm.fr
authors.forename: N
authors.initials: N
authors.lastname: Schleinitz
authors.forename: P
authors.initials: P
authors.lastname: Poullin
authors.forename: L
authors.initials: L
authors.lastname: Camoin
authors.forename: V
authors.initials: V
authors.lastname: Veit
authors.forename: E
authors.initials: E
authors.lastname: Bernit
authors.forename: K
authors.initials: K
authors.lastname: Mazodier
authors.forename: P
authors.initials: P
authors.lastname: Lefèvre
authors.forename: F
authors.initials: F
authors.lastname: Dignat-George
authors.forename: G
authors.initials: G
authors.lastname: Kaplanski
authors.forename: J M
authors.initials: JM
authors.lastname: Durand
authors.forename: J R
authors.initials: JR
authors.lastname: Harlé
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D003937:Diagnosis, Differential; D006801:Humans; D010951:Plasma Exchange; D010975:Platelet Aggregation Inhibitors / Q000627:therapeutic use; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000453:epidemiology / Q000503:physiopathology / Q000628:therapy*
chemical_list: D000305:Adrenal Cortex Hormones; D010975:Platelet Aggregation Inhibitors