title: [The clinical studies of 51 patients with thrombotic thrombocytopenic purpura].
abstract: OBJECTIVE
  To comprehensively analyze the clinical characteristics, treatment strategies and outcome of patients with thrombotic thrombocytopenic purpura (TTP).
  
  
  METHODS
  A retrospective survey of 51 TTP patients confirmed in our database. Relevant statistical analyzes were performed by GraphPad Prism 5 software.
  
  
  RESULTS
  51 cases of patients with acquired TTP were identified as idiopathic TTP. In our study, only 18 cases (35.29%) had typical pentalogy of TTP, where thrombocytopenia (100.00%), microangiopathic hemolytic anemia (92.16%) and neurologic abnormalities (88.24%) were more common than fever (72.55%) and renal abnormalities (70.59%). Plasma ADAMTS13 activity was detected in 37 patients with TTP with ADAMTS13 deficiency confirmed in 31 patients (83.78%). Plasma exchange with response of 72.3% was still the preferred strategy in TTP with individuation. Among 36 survival TTP patients, 8 patients (22.22%) relapsed. 15 patients (29.41%) died in our study. The mean ages of responders and deaths were of (37.5±14.5) and (50.1±18.9) respectively; whereas total bilirubin level of responders and deaths were of (43.3±23.5)μmol/L and (63.7±39.7) μmol/L respectively, the differences were statistically significant. Conversely, body temperature, WBC, HGB, PLT, serum creatinine and LDH showed no significant differences (P>0.05).
  
  
  CONCLUSIONS
  The diagnosis of TTP was based on comprehensive analysis of clinical manifestations. Plasma ADAMTS13 activity test had a higher clinical practical value. The therapeutic alliance with corticosteroids, immunosuppressive agents and Rituximab significantly improved its outcome. The age and high total bilirubin level at onset were associated with less sensitive to plasmapheresis and poor prognosis.
authors.affiliation: Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.
authors.forename: Ling
authors.initials: L
authors.lastname: Sun
authors.affiliation: Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.
authors.forename: Ziqiang
authors.initials: Z
authors.lastname: Yu
authors.affiliation: Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.
authors.forename: Yunwen
authors.initials: Y
authors.lastname: Bu
authors.affiliation: Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.
authors.forename: Jian
authors.initials: J
authors.lastname: Su
authors.affiliation: Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.
authors.forename: Chaorong
authors.initials: C
authors.lastname: Wang
authors.affiliation: Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.
authors.forename: Lijuan
authors.initials: L
authors.lastname: Cao
authors.affiliation: Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.
authors.forename: Xia
authors.initials: X
authors.lastname: Bai
authors.affiliation: Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.
authors.forename: Zhaoyue
authors.initials: Z
authors.lastname: Wang
authors.affiliation: Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Suzhou 215006, China.
authors.forename: Changgeng
authors.initials: C
authors.lastname: Ruan
mesh_terms: D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis* / Q000628:therapy*; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human