title: Treatment outcomes in patients with adult thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.
abstract: BACKGROUND
  Plasma treatment has improved the outcomes in adults with thrombotic thrombocytopenic purpura (TTP)-hemolytic uremic syndrome (HUS). We reviewed our experience in treating unselected patients to determine the clinical outcomes and to evaluate the treatments given in addition to plasma.
  
  
  METHODS
  A chart review of all cases of TTP and HUS in adults treated at the Toronto (Ontario) Hospital, the largest treatment center for adults with TTP-HUS in the province of Ontario, was conducted.
  
  
  RESULTS
  Sixty-seven episodes of TTP-HUS in 52 consecutive adult patients were treated during a 12-year period. Plasma was the primary form of therapy, and most patients received plasma exchange. A complete hematologic remission was achieved in 65 of 67 episodes; however, two patients in remission were brain-dead. The time to complete remission varied from 3 to 58 days (median, 13 days). The death rate during the acute illness was 8%. Long-term sequelae included relapses, persisting renal impairment, hepatitis, and transfusion-associated acquired immunodeficiency syndrome. Relapses occurred in 21% of patients during a median follow-up of 1.1 years (range, 0.1 to 18 years). Analyses of the treatment given in addition to plasma did not demonstrate a significant benefit in terms of reducing the illness duration, mortality, or long-term sequelae.
  
  
  CONCLUSIONS
  While most patients recovered from TTP-HUS, deaths still occurred and many patients suffered long-term complications. The role of the treatments given in addition to plasma is uncertain.
authors.affiliation: Department of Hematology, Toronto (Ontario) Hospital.
authors.forename: C P
authors.initials: CP
authors.lastname: Hayward
authors.forename: D M
authors.initials: DM
authors.lastname: Sutton
authors.forename: W H
authors.initials: WH
authors.lastname: Carter
authors.forename: E D
authors.initials: ED
authors.lastname: Campbell
authors.forename: J G
authors.initials: JG
authors.lastname: Scott
authors.forename: W H
authors.initials: WH
authors.lastname: Francombe
authors.forename: K H
authors.initials: KH
authors.lastname: Shumak
authors.forename: M A
authors.initials: MA
authors.lastname: Baker
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D017707:Erythrocyte Transfusion; D005260:Female; D006463:Hemolytic-Uremic Syndrome / Q000150:complications / Q000209:etiology / Q000628:therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010951:Plasma Exchange; D011247:Pregnancy; D011250:Pregnancy Complications, Hematologic / Q000628:therapy; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000628:therapy*; D012008:Recurrence; D017582:Renal Replacement Therapy; D013156:Splenectomy; D016896:Treatment Outcome