title: Clinical experience in treatment of thrombotic thrombocytopenic purpura--hemolytic uremic syndrome with 28 patients.
abstract: BACKGROUND
  Neither optimal treatment nor significance of ADAMTS13 (A Desintegrin And Metalloprotease with ThromboSpondin type 1 repeats) activity for diagnosis and therapy of thrombotic thrombocytopenic purpura (TTP) and hemolytic-uremic syndrome (HUS) have not been defined yet. The aim of the report is to analyze response to different volumes of plasma exchange (PE), and relationship to ADAMTS13.
  
  
  METHODS
  28 patients clinically diagnosed with idiopathic TTP (n = 18), secondary TTP (n = 4), atypical HUS (n = 3) and typical HUS (n = 3) manifested 31 acute episodes. Patients were treated with PE in 26, and with plasma transfusion in 5 episodes with additional different therapies.
  
  
  RESULTS
  PE volumes were as follows: 1 in 7, 1.5 in 3, 2 in 14, and intensifying schedule (1 to 1.5) in 2 episodes. Procedure number was lower in patients treated with 2 and 1.5 (p = 0.019) than in those treated with 1 volume exchange and PE intensifying, respectively (p = 0.010). PE response rate was 25/26 (96.15%). Exacerbation frequency was higher in idiopathic TTP patients (3/19) treated with 1 compared with patients treated with > 1 volume exchange (p = 0.003). Survival rate was 25/28 (89.29%). ADAMTS13 activity was reduced in 22 with severe deficiency in 14 patients.
  
  
  CONCLUSIONS
  Patients responded to different treatments regardless of ADAMTS13 activity, requiring less PEs with larger volume exchanges.
authors.forename: Dragica
authors.initials: D
authors.lastname: Vuclić
authors.forename: Zoran
authors.initials: Z
authors.lastname: Rajić
authors.forename: Nebojsa
authors.initials: N
authors.lastname: Savić
authors.forename: Danijela
authors.initials: D
authors.lastname: Miković
authors.forename: Zivko
authors.initials: Z
authors.lastname: Budisin
authors.forename: Nebojsa M
authors.initials: NM
authors.lastname: Antonijević
authors.forename: Slobodan
authors.initials: S
authors.lastname: Obradović
authors.forename: Dragana
authors.initials: D
authors.lastname: Jevtić
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Bettoni
authors.forename: Gloria
authors.initials: G
authors.lastname: Casoli
authors.forename: Flora
authors.initials: F
authors.lastname: Peyvandi
mesh_terms: D051722:ADAM Proteins / Q000097:blood* / Q000172:deficiency / Q000276:immunology; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000368:Aged; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000201:enzymology / Q000628:therapy*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D010949:Plasma; D010951:Plasma Exchange; D011241:Prednisone / Q000627:therapeutic use; D011697:Purpura, Thrombotic Thrombocytopenic / Q000201:enzymology / Q000628:therapy*; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D000893:Anti-Inflammatory Agents; D001323:Autoantibodies; D007155:Immunologic Factors; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human; D011241:Prednisone; D008775:Methylprednisolone