Laboratory testing for ADAMTS13: Utility for TTP diagnosis/exclusion and beyond

Am J Hematol. 2021 Aug 1;96(8):1049-1055. doi: 10.1002/ajh.26241. Epub 2021 May 31.


The metalloproteinase ADAMTS13 (a disintegrin with a thrombospondin type 1 motif, member 13), also known as VWF (von Willebrand factor) protease, may be assessed in a vast array of clinical conditions. Notably, a severe deficiency of ADAMTS13 characterizes TTP (thrombotic thrombocytopenic purpura), a rare but potentially fatal disorder associated with thrombosis due to accumulation of prothrombotic ultra-large VWF multimers. Although prompt identification/exclusion of TTP can be facilitated by rapid ADAMTS13 testing, the most commonly utilized assays are based on ELISA (enzyme linked immunosorbent assay) and require long turnaround time and have relatively limited throughput. Nevertheless, several rapid ADAMTS13 assays are now available, at least in select geographies. The current mini-review discusses these issues, as well as the potential utility of ADAMTS13 testing in a range of other conditions, including coronavirus disease 2019 (COVID-19).

Publication types

  • Review

MeSH terms

  • ADAMTS13 Protein / blood*
  • ADAMTS13 Protein / deficiency
  • ADAMTS13 Protein / immunology
  • Autoantibodies / blood
  • Autoantibodies / immunology
  • COVID-19 / blood
  • COVID-19 / complications*
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Fluorescence Resonance Energy Transfer
  • Humans
  • Luminescent Measurements
  • Male
  • Multicenter Studies as Topic
  • Pre-Eclampsia / diagnosis
  • Pre-Eclampsia / enzymology
  • Predictive Value of Tests
  • Pregnancy
  • Purpura, Thrombotic Thrombocytopenic / blood
  • Purpura, Thrombotic Thrombocytopenic / diagnosis*
  • Purpura, Thrombotic Thrombocytopenic / enzymology
  • Purpura, Thrombotic Thrombocytopenic / etiology
  • Recombinant Proteins / metabolism
  • SARS-CoV-2*
  • Sensitivity and Specificity
  • von Willebrand Diseases / diagnosis
  • von Willebrand Diseases / enzymology
  • von Willebrand Factor / metabolism


  • Autoantibodies
  • Recombinant Proteins
  • von Willebrand Factor
  • ADAMTS13 Protein
  • ADAMTS13 protein, human