title: [Management of thrombotic thrombocytopenic purpura in two French centers: A series of 27 patients].
abstract: BACKGROUND
  Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy due to platelet microthrombosis involved in multiple systems associated with multiple organ dysfunctions and often severe disease course.
  
  
  METHODS
  In order to enhance the understanding of TTP, the clinical features, laboratory characteristics, treatment and outcome of 27 patients with TTP were retrospectively analyzed and investigated in two centres (Annecy and Grenoble).
  
  
  RESULTS
  All the 27 patients presented with haemolytic anemia and decreased platelet counts. Eight patients had fever. Thirteen patients had kidney involvement including proteinuria and renal function abnormalities. Eighteen patients had neurological manifestations. Association of the 5 characteristic features of TTP was rarely found (11%) which could lead to diagnosis delay. The von Willebrand factor-cleaving protease (ADAMTS13) activity was less than 10% in all patients. At the same time, plasma ADAMTS13 inhibitors were detected in 20 patients out of the 27. After treatment with plasma exchange, glucocorticoid and rituximab, 23 patients (85%) achieved complete remission. Four patients died, in which two cases were secondary to late diagnosis.
  
  
  CONCLUSIONS
  TTP is a thrombotic microangiopathy often associated with multiple organ dysfunctions. Salvage therapy, based on some studies experiences and discussed with the Reference Center, could help getting answers in most severe cases. Awareness of physicians is an important step to further limit any delay in medical care, and to further improve the prognosis of patients with TTP.
authors.affiliation: Service de médecine interne, hôpital Michallon, CHU de Grenoble, boulevard de la Chantourne, 38700 La Tronche, France. Electronic address: aderoux@chu-grenoble.fr.
authors.forename: A
authors.initials: A
authors.lastname: Deroux
authors.affiliation: Service de réanimation polyvalente, centre hospitalier d'Annecy, 74000 Annecy, France.
authors.forename: M
authors.initials: M
authors.lastname: Sirodot
authors.affiliation: Service d'hématologie, centre hospitalier d'Annecy, 74000 Annecy, France.
authors.forename: N
authors.initials: N
authors.lastname: Daguindau
authors.affiliation: Service d'hémato-biologie, CHU de Grenoble, 38000 Grenoble, France.
authors.forename: C
authors.initials: C
authors.lastname: Barro
authors.affiliation: Service d'hématologie, hôpital Saint-Antoine, AP-HP, Paris, France; Centre de référence des microangiopathies thrombotiques, hôpital Saint-Antoine, AP-HP, Paris, France; UPMC Sorbonne universités, université Paris 06, 75005 Paris, France; Inserm U1009, institut Gustave-Roussy, Villejuif, France.
authors.forename: P
authors.initials: P
authors.lastname: Coppo
authors.affiliation: Service de médecine interne, hôpital Michallon, CHU de Grenoble, boulevard de la Chantourne, 38700 La Tronche, France; Université Joseph-Fourier, Grenoble I, 38000 Grenoble, France.
authors.forename: L
authors.initials: L
authors.lastname: Bouillet
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D005260:Female; D005602:France / Q000453:epidemiology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange / Q000706:statistics & numerical data; D011379:Prognosis; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D000069283:Rituximab
keywords: ADAMTS13; Microangiopathie thrombotique; Plasmatic exchange; Purpura thrombotique thrombocytopénique; Rituximab; Thrombotic microangiopathy; Thrombotic thrombocytopenic purpura; Échanges plasmatiques