title: Cyclosporin A therapy on idiopathic thrombotic thrombocytopenic purpura in the relapse setting: two case reports and a review of the literature.
abstract: BACKGROUND
  Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease, characterized by microangiopathic hemolytic anemia, thrombocytopenia, fever, neurologic disturbances, and renal failure. Plasma therapy has dramatically improved prognosis of TTP, whereas recurrent acute episodes still occur in approximately 40% of patients. Moreover, patients with acquired ADAMTS13 deficiency, which is a significant factor for relapse, may require additional immunosuppressive treatment to get a durable remission.
  
  
  METHODS
  We hereby report two patients with a history of relapsed idiopathic TTP, who both received cyclosporin A (CSA) as a prophylactic manner after the remission was achieved. We also discuss the efficacy of CSA in patients with relapsed idiopathic TTP with a review of the published literature.
  
  
  RESULTS
  Under CSA therapy, both patients maintained their clinical remission state, and the ADAMTS13 levels were normalized.
  
  
  CONCLUSIONS
  To conclude, CSA therapy may be useful for the prevention of relapsed idiopathic TTP in patients with a history of frequent relapses.
authors.affiliation: Department of Nephrology, Bakirkoy Dr Sadi Konuk Teaching Hospital, Istanbul, Turkey. murvetyilmaz@hotmail.com
authors.forename: Murvet
authors.initials: M
authors.lastname: Yilmaz
authors.forename: Ahmet Emre
authors.initials: AE
authors.lastname: Eskazan
authors.forename: Abdulkadir
authors.initials: A
authors.lastname: Unsal
authors.forename: Hakan
authors.initials: H
authors.lastname: Taninmis
authors.forename: Ekrem
authors.initials: E
authors.lastname: Kara
authors.forename: Mustafa
authors.initials: M
authors.lastname: Cetiner
authors.forename: Burhan
authors.initials: B
authors.lastname: Ferhanoglu
mesh_terms: D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000328:Adult; D016572:Cyclosporine / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D011697:Purpura, Thrombotic Thrombocytopenic / Q000188:drug therapy*; D012008:Recurrence
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human