title: Clinical utility of ADAMTS-13 testing in suspected thrombotic microangiopathy:  an audit of ADAMTS-13 activity assay requests in routine practice from a tertiary hospital.
abstract: OBJECTIVE
  Differentiation between thrombotic thrombocytopenic purpura (TTP) and other microangiopathic haemolytic anaemia (MAHA) processes can be difficult. Since the documentation of ADAMTS-13 deficiency in TTP, several ADAMTS-13 activity assays have been developed for use in the diagnosis of TTP and/or other microangiopathic disorders. We reviewed the clinical utility of ADAMTS-13 activity testing in suspected TTP, as used in routine clinical practice in a tertiary referral hospital.
  
  
  METHODS
  All requests for ADAMTS-13 activity levels performed at our institution after introduction of the assay were retrospectively audited with respect to clinical diagnosis and results.
  
  
  RESULTS
  In total 57 individual patients were tested, of whom only 46% had a MAHA process. Severe ADAMTS-13 deficiency was present in five TTP patients and in one patient with fulminant hepatic failure.
  
  
  CONCLUSIONS
  Our experience suggests that severely reduced levels are relatively specific for TTP, but may also occur in fulminant hepatic failure. Patients without MAHA may have reduced ADAMTS-13 activity and there is significant overlap in the range of ADAMTS-13 activity seen in non-TTP MAHA diagnoses. This supports the observation that outside the diagnosis and (possible) follow-up of suspected idiopathic TTP, the ADAMTS-13 activity assay has limited clinical utility. Further education about the role of ADAMTS-13 activity testing is needed.
authors.affiliation: Pathology Queensland, Department of Haematology, Royal Brisbane and Women's Hospital, Herston, Queensland, Australia.
authors.forename: Dominique G
authors.initials: DG
authors.lastname: Garizio
authors.forename: Urs
authors.initials: U
authors.lastname: Wilgen
authors.forename: Bronwyn A
authors.initials: BA
authors.lastname: Williams
authors.forename: Glen A
authors.initials: GA
authors.lastname: Kennedy
mesh_terms: D051722:ADAM Proteins / Q000097:blood* / Q000378:metabolism; D000071120:ADAMTS13 Protein; D000743:Anemia, Hemolytic / Q000097:blood / Q000175:diagnosis* / Q000378:metabolism; D003937:Diagnosis, Differential; D031541:Fluorescence Resonance Energy Transfer; D006801:Humans; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood / Q000175:diagnosis* / Q000378:metabolism; D012189:Retrospective Studies; D062606:Tertiary Care Centers; D057049:Thrombotic Microangiopathies / Q000097:blood / Q000175:diagnosis* / Q000378:metabolism
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human