title: Safety and efficacy of cryoprecipitate-poor plasma as a replacement fluid for therapeutic plasma exchange in thrombotic thrombocytopenic purpura: a single center retrospective evaluation.
abstract: BACKGROUND
  Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy caused by decreased activity of ADAMTS13, resulting in reduced clearance of ultralarge von Willebrand factor (VWF) multimers. Treatment of TTP is therapeutic plasma exchange (TPE) with replacement with fresh frozen plasma (FFP). Cryoprecipitate-poor plasma (CPP) is a plasma product with lower concentrations of large VWF multimers, and similar amounts of ADAMTS13. CPP is regarded as at least as efficacious as FFP in TTP but evidence of additional benefits has not been demonstrated. Furthermore, there are limited data on the frequency of adverse events associated with CPP.
  
  
  METHODS
  In our center, the choice between CPP and FFP is performed before the 1st TPE session at the physicians' discretion. Here, we retrospectively evaluated the efficacy and safety of CPP based on the number of sessions, volume of plasma exposure, frequency of exacerbations/relapses, and adverse events.
  
  
  RESULTS
  Fourteen patients with newly diagnosed TTP were included in this analysis. The proportion of CPP:FFP use was 5:9. There were no significant differences in age, gender, initial hemoglobin, platelet count, LDH, or etiology of TTP between groups. We observed a trend toward a higher number of TPE sessions and higher plasma exposure in CPP, compared to FFP-treated patients. Acute exacerbations were more frequent among patients treated with CPP (OR 26.6; 95%CI 1.01-703.51; P = 0.03). Mild allergic reactions were the most common treatment-related adverse event in both groups.
  
  
  CONCLUSIONS
  Our data suggest that CPP should not be used as 1st line treatment for newly diagnosed TTP patients.
authors.affiliation: Hematology and Hemotherapy Center, University of Campinas, Campinas, São Paulo, Brazil.
authors.forename: Bianca
authors.initials: B
authors.lastname: Stefanello
authors.forename: Erich Vinícius
authors.initials: EV
authors.lastname: De Paula
authors.forename: Fernanda
authors.initials: F
authors.lastname: Andrade Orsi
authors.forename: Jose Francisco
authors.initials: JF
authors.lastname: Comenalli Marques
authors.forename: Eduardo
authors.initials: E
authors.lastname: Gasparotto Roveri
authors.forename: Marina
authors.initials: M
authors.lastname: Pereira Colella
authors.forename: Margareth
authors.initials: M
authors.lastname: Castro Ozelo
authors.forename: Joyce
authors.initials: J
authors.lastname: Maria Annichino-Bizzacchi
authors.forename: Marcelo
authors.initials: M
authors.lastname: Addas-Carvalho
mesh_terms: D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000328:Adult; D023341:Chills / Q000209:etiology; D005169:Factor VIII; D005260:Female; D005334:Fever / Q000209:etiology; D005340:Fibrinogen; D005767:Gastrointestinal Diseases / Q000209:etiology; D006801:Humans; D006967:Hypersensitivity / Q000209:etiology; D008297:Male; D008875:Middle Aged; D010949:Plasma; D010951:Plasma Exchange / Q000009:adverse effects / Q000379:methods*; D011697:Purpura, Thrombotic Thrombocytopenic / Q000628:therapy*; D012008:Recurrence; D012189:Retrospective Studies; D055815:Young Adult
chemical_list: C026912:cryoprecipitate coagulum; D005169:Factor VIII; D005340:Fibrinogen; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human
keywords: ADAMTS13; cryosupernatant; fresh frozen plasma; microangiopathy; plasma exchange