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Incidence of thrombotic microangiopathies in Quebec: insight from a laboratory centralizing ADAMTS-13 testing
Clémence Merlen1, Emmanuelle Pépin1,2, Ousmane Barry1
1Division of Hematology-Oncology, CHU Sainte-Justine, Université de Montréal, 3175, chemin de la Côte-Ste-Catherine, Montreal, QC, H3T 1C5, Canada.
Background:
Thrombotic microangiopathies (TMA) are serious medical conditions requiring a prompt diagnosis to adapt treatment. The determination of ADAMTS-13 activity enables discriminating thrombotic thrombocytopenic purpura (TTP) from other forms of TMA. The purpose of this study was to provide an estimate of the incidence of TTP and TMA in the Canadian Quebec province using data collected from a laboratory centralizing ADAMTS-13 testing for the whole province.
Results:
From 2012 to 2019, 846 patients were evaluated for plasma ADAMTS-13 activity due to a suspicion of TMA. TTP was identified in 147 patients. Of these, 118 patients with a median age of 51.5 years and a male-female ratio of 1:1.4 had their first episode of TTP during the study period. The number of ADAMTS-13 tests performed and the number of patients with suspected TMA increased annually by 19% and 21% respectively. While the incidence of non-TTP TMA increased annually, that for TTP remained unchanged. This averaged 10.2 (95% CI 5.9-14.4) per million persons per year for suspected non-TTP TMA and 1.8 (95% CI 1.3-2.4) for confirmed TTP. The incidence rate of TMA other than TTP was higher in the age group 70-79 years (21.8; 95% CI 5.4-38.1) for females and in the age group 80-89 years (24.4; 95% CI 7.2-41.7) for males compared to other age groups. The incidence rate of TTP was higher in the age group 40-49 years (4.0; 95% CI 2.0-5.9) for women and in the age group 60-69 years (3.4; 95% CI 1.1-5.6) for men compared to other age groups.
Conclusion:
The analysis of centralized data measuring ADAMTS-13 activity allowed us to adequately establish the incidence rate and demographic characteristics of TMA, particularly TTP, in Quebec. TTP incidence remained stable while suspected non-TTP TMA steadily increased from 2012 to 2019.
Insights
Thrombotic microangiopathies (TMA) incidence in Quebec increased, with suspected non-TTP TMA rising annually. However, the incidence of thrombotic thrombocytopenic purpura (TTP) remained stable between 2012 and 2019.
Area of Science:
- Hematology
- Epidemiology
- Clinical Diagnostics
Background:
- Thrombotic microangiopathies (TMA) are critical conditions necessitating rapid diagnosis and treatment.
- ADAMTS-13 activity testing is crucial for differentiating thrombotic thrombocytopenic purpura (TTP) from other TMAs.
Purpose of the Study:
- To estimate the incidence of TTP and other TMAs in Quebec, Canada.
- To analyze demographic characteristics associated with TTP and TMA incidence.
Main Methods:
- Utilized centralized laboratory data for ADAMTS-13 activity testing from 2012 to 2019.
- Evaluated 846 patients with suspected TMA, identifying 147 with TTP.
Main Results:
- The incidence of suspected non-TTP TMA averaged 10.2 per million person-years and increased annually.
- The incidence of confirmed TTP averaged 1.8 per million person-years and remained stable.
- TTP incidence was higher in women aged 40-49 and men aged 60-69; non-TTP TMA incidence varied by age and sex.
Conclusions:
- Centralized ADAMTS-13 testing data provide reliable TMA and TTP incidence estimates in Quebec.
- While TTP incidence was stable, other TMA forms showed an increasing trend from 2012 to 2019.

