title: Twice-daily therapeutical plasma exchange-based salvage therapy in severe autoimmune thrombotic thrombocytopenic purpura: the French TMA Reference Center experience.
abstract: BACKGROUND
  Daily therapeutic plasma exchange (TPE) and rituximab improved thrombotic thrombocytopenic purpura (TTP) prognosis. In the more severe cases, salvage therapies including twice-daily TPE and/or cyclophosphamide may be proposed and require evaluation.
  
  
  METHODS
  TTP was defined as a thrombotic microangiopathy (TMA) with severe (<10%) acquired ADAMTS13 deficiency. Among patients included in the French Reference Center for TMA registry, we considered those with a severe disease (i.e., unresponsive to daily TPE and rituximab) who received twice-daily TPE.
  
  
  RESULTS
  Nineteen of 289 (6.6%) patients with TTP were treated by twice-daily TPE between 2008 and 2014. Twice-daily TPE was associated with rituximab in 16 cases. The median duration of twice-daily TPE treatment was 3 d (2-22 d). In 6 patients (31.6%), additional treatments (mainly pulses of cyclophosphamide) were performed because of a persistently refractory disease (4 cases) or an exacerbation (2 cases), despite twice-daily TPE. Only one patient (5.3%) died. The other 18 achieved a durable complete remission 25.5 d (13-68 d) after the first TPE. The median follow-up was 14.4 months (7 d-45 months).
  
  
  CONCLUSIONS
  Twice-daily TPE may be an efficient strategy in the more severe TTP patients with a short-term life-threatening disease that could overcome their poor prognosis.
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Paris, France.|Service de Réanimation, Centre Hospitalier Métropole Savoie, Chambéry, France.
authors.forename: Myriam
authors.initials: M
authors.lastname: Soucemarianadin
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Paris, France.|Inserm U1096, Rouen, France.|Service de Médecine Interne, CHU Charles Nicolle, Rouen, France.
authors.forename: Ygal
authors.initials: Y
authors.lastname: Benhamou
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Paris, France.|Service de Néphrologie Transplantation Dialyse, Centre Hospitalier Universitaire, Bordeaux, France.
authors.forename: Yahsou
authors.initials: Y
authors.lastname: Delmas
authors.affiliation: Sorbonne Université, UPMC Univ Paris 06, Paris, France.|Service de Réanimation Médicale, CHU Saint-Antoine, Paris, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Pichereau
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Paris, France.|Sorbonne Université, UPMC Univ Paris 06, Paris, France.|Service de Réanimation Médicale, CHU Saint-Antoine, Paris, France.
authors.forename: Eric
authors.initials: E
authors.lastname: Maury
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Paris, France.|Service de Réanimation Polyvalente, Hôpital Cochin, Paris, France.|Université Paris 5, Paris, France.
authors.forename: Frédéric
authors.initials: F
authors.lastname: Pène
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Paris, France.|Service de néphrologie-immunologie clinique, hôpital Bretonneau, Tours, France.|EA 4245, université François-Rabelais 2, boulevard Tonnellé, Tours, France.
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Halimi
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Paris, France.|Service de Néphrologie - Médecine Interne, Hôpital Sud, Amiens, France.
authors.forename: Claire
authors.initials: C
authors.lastname: Presne
authors.affiliation: Service de Réanimation, Centre Hospitalier Métropole Savoie, Chambéry, France.
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Thouret
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Paris, France.|Service d'Hématologie Biologique, Hôpital Lariboisière, Paris, France.|Univ. Paris Diderot, Sorbonne Paris Cité, Paris, France.
authors.forename: Agnès
authors.initials: A
authors.lastname: Veyradier
authors.affiliation: Centre de Référence des Microangiopathies Thrombotiques, Hôpital Saint-Antoine, Paris, France.|Sorbonne Université, UPMC Univ Paris 06, Paris, France.|Inserm U1009, Institut Gustave Roussy, Villejuif, France.|Service d'Hématologie, Hôpital Saint-Antoine, Paris, France.
authors.forename: Paul
authors.initials: P
authors.lastname: Coppo
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D005260:Female; D005602:France; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange / Q000379:methods; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000276:immunology / Q000628:therapy*; D016879:Salvage Therapy; D012720:Severity of Illness Index; D016896:Treatment Outcome; D055815:Young Adult
keywords: ADAMTS13; autoimmune thrombotic thrombocytopenic purpura; twice-daily therapeutic plasma exchange