ArticleResearch and practice in thrombosis and haemostasis2025
Standard cardiovascular risk prediction scores underestimate risk in immune-mediated thrombotic thrombocytopenic purpura survivors.
Article in Research and practice in thrombosis and haemostasis, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
1 citing paper in PubMed.
- Assessment of cardiovascular disease in immune thrombotic thrombocytopenic purpura.Research and practice in thrombosis and haemostasis · 2026Article
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7 authors.
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Abstract
Background: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) is a rare hematologic disorder with improved survival due to advancements in treatment. However, long-term cardiovascular morbidity and mortality remain significant. Established cardiovascular risk calculators, such as the 2008 Framingham Heart Study (FHS) global cardiovascular disease (CVD) and the American College of Cardiology/American Heart Association (ACC/AHA) atherosclerotic CVD (ASCVD) risk estimators, may not adequately account for the elevated and unique cardiovascular risks in iTTP survivors. Objectives: To evaluate the discrimination and calibration of the ACC/AHA ASCVD and FHS global CVD models in predicting major adverse cardiovascular events (MACEs) among iTTP survivors. Methods: This retrospective study analyzed 135 iTTP survivors from Johns Hopkins University (1994-2024). Presence of MACEs, including myocardial infarction, stroke, and cardiac revascularization, was the primary outcome and was assessed during clinical remission. Discriminatory ability of the model was assessed using c-statistics, while calibration was evaluated with Hosmer-Lemeshow tests and calibration plots. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were also calculated. Results: MACEs occurred in 37.8% of the cohort over a median follow-up of 3.8 years. The ASCVD and FHS models demonstrated poor discrimination (c-statistics, 0.54 and 0.52, respectively) and poor calibration, with observed MACE rates exceeding predicted probabilities (Hosmer-Lemeshow Conclusion: Standard cardiovascular risk models inadequately predict MACE risk in iTTP survivors, underscoring the need for tailored tools that incorporate iTTP-specific factors to improve cardiovascular risk stratification and management.
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