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

Area of Science:

• Hematology
• Clinical Diagnostics
• Enzymology

Background:

• ADAMTS13 (a disintegrin with a thrombospondin type 1 motif, member 13) is a metalloproteinase, also known as von Willebrand factor (VWF) protease.
• Severe ADAMTS13 deficiency is the hallmark of thrombotic thrombocytopenic purpura (TTP), a life-threatening condition characterized by thrombosis from ultra-large VWF multimers.

Purpose of the Study:

• To review the clinical utility of ADAMTS13 testing across various conditions.
• To discuss the advantages of rapid ADAMTS13 assays over traditional enzyme-linked immunosorbent assays (ELISA).
• To explore the potential role of ADAMTS13 testing in diseases such as coronavirus disease 2019 (COVID-19).

Main Methods:

• Review of current literature on ADAMTS13 assays and clinical applications.
• Comparison of traditional ELISA-based assays with newer rapid ADAMTS13 testing methodologies.
• Discussion of the diagnostic and prognostic value of ADAMTS13 activity measurement.

Main Results:

• Traditional ELISA assays for ADAMTS13 have long turnaround times and limited throughput.
• Several rapid ADAMTS13 assays are now available, facilitating quicker diagnosis of TTP.
• ADAMTS13 testing shows potential utility beyond TTP, including in COVID-19.

Conclusions:

• Rapid ADAMTS13 testing is crucial for prompt TTP diagnosis and management.
• Advancements in assay technology are improving the accessibility and efficiency of ADAMTS13 assessment.
• Further research is warranted to fully elucidate the role of ADAMTS13 in diverse clinical settings.