Division of Hematology-Oncology, CHU Sainte-Justine, Université de Montréal, 3175, chemin de la Côte-Ste-Catherine, Montreal, QC, H3T 1C5, Canada.
Department of Clinical Laboratory Medicine, OPTILAB Montréal-CHU Sainte-Justine, Montreal, QC, Canada.
Orphanet J Rare Dis. 2022 Aug 4;17(1):308. doi: 10.1186/s13023-022-02409-3.
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.
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.
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.
血栓性微血管病（TMA）是一种严重的医学病症，需要迅速诊断以调整治疗方法。测定 ADAMTS-13 活性有助于鉴别血栓性血小板减少性紫癜（TTP）与其他形式的 TMA。本研究旨在利用全省集中 ADAMTS-13 检测数据，估算加拿大魁北克省 TTP 和 TMA 的发病率。
2012 年至 2019 年，因疑似 TMA 而对 846 例患者进行了血浆 ADAMTS-13 活性评估。共发现 147 例 TTP，其中 118 例患者年龄中位数为 51.5 岁，男女比例为 1:1.4，在研究期间首次发生 TTP。ADAMTS-13 检测次数和疑似 TMA 患者数量每年分别以 19%和 21%的速度增长。非 TTP TMA 的发病率呈逐年上升趋势，而 TTP 则保持不变。疑似非 TTP TMA 的年发病率为 10.2（95%CI 5.9-14.4）/百万人，确诊 TTP 的年发病率为 1.8（95%CI 1.3-2.4）/百万人。70-79 岁女性（21.8；95%CI 5.4-38.1）和 80-89 岁男性（24.4；95%CI 7.2-41.7）非 TTP TMA 的发病率高于其他年龄组。40-49 岁女性（4.0；95%CI 2.0-5.9）和 60-69 岁男性（3.4；95%CI 1.1-5.6）TTP 的发病率高于其他年龄组。
对集中测定 ADAMTS-13 活性的数据进行分析，使我们能够充分确定魁北克省 TMA，尤其是 TTP 的发病率和人口统计学特征。TTP 的发病率保持稳定，而疑似非 TTP TMA 从 2012 年到 2019 年稳步增加。
