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. 2022 Apr;63(4):214-218.
doi: 10.11622/smedj.2020093. Epub 2020 Jun 26.

Reference range for ADAMTS13 antigen, activity and anti-ADAMTS13 antibody in the healthy adult Singapore population

Allison Ching Yee Tso  1 , Christina Lai Lin Sum  2 , Kiat Hoe Ong  1
Affiliations

Reference range for ADAMTS13 antigen, activity and anti-ADAMTS13 antibody in the healthy adult Singapore population

Allison Ching Yee Tso et al. Singapore Med J. 2022 Apr.

Abstract

Introduction: ADAMTS13 (a disintegrin-like and metalloproteinase with a thrombospondin Type 1 motif, member 13) plays a fundamental role in the regulation of haemostasis and thrombosis. Its deficiency leads to an accumulation of ultra-large von Willebrand multimers, inducing spontaneous platelet aggregation, thrombosis in the microvasculature, and thrombotic thrombocytopenic purpura (TTP), a condition with 90% mortality when left untreated. Prompt quantification of ADAMTS13 antigen, activity and autoantibody plays a crucial role in the diagnosis and management of TTP and can help differentiate it from other thrombotic microangiopathies (TMAs). Reference ranges for ADAMTS13 are generally derived from Caucasian patients. Given that polymorphism in the ADAMTS13 gene can be associated with variable ADAMTS13 levels, we aimed to establish the first reference range in Singapore and provide a crucial laboratory test for institutions here and elsewhere.

Methods: 150 healthy voluntary donors (75 men, 75 women) aged 21-60 years, with an ethnic mix mirroring Singapore's population profile, were recruited. ADAMTS13 antigen, activity and autoantibody levels were measured using the fluorescence resonance energy transfer-vWF73 and enzyme-linked immunosorbent assay methodologies.

Results: Levels (activity 0.65-1.79 IU/mL, antigen 0.36-1.17 IU/mL, autoantibody 1.4-12.5 U/mL) were not statistically different between the genders and various age groups.

Conclusion: TTP and TMAs are encountered in a wide range of specialties. The availability of new assays in Singapore will aid clinicians in the timely management of these conditions. Standardising reference ranges established for Singapore against World Health Organization standards allows harmonisation of measurements between laboratories and for future research collaborations.

Keywords: ADAMTS13; microangiopathic haemolytic anaemia; thrombotic thrombocytopenic purpura.

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Figures

Fig. 1
Fig. 1
Charts show ADAMTS13 activity and antigen levels according to (a) age group and (b) ethnicity. ADAMTS13: a disintegrin-like and metalloprotease with thrombospondin Type 1 motif, member 13
Fig. 2
Fig. 2
Charts show ADAMTS13 autoantibody levels according to (a) age group and (b) ethnicity. ADAMTS13: a disintegrin-like and metalloprotease with thrombospondin Type 1 motif, member 13
See this image and copyright information in PMC

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