标准心血管风险预测评分低估了免疫介导的血栓性血小板减少性紫癜幸存者的风险

IF 3.4 3区医学 Q2 HEMATOLOGY

Research and Practice in Thrombosis and Haemostasis Pub Date : 2025-08-01 DOI:10.1016/j.rpth.2025.103005

Binish Javed , Jenna Brown , Jay Meade , Vijay Nambi , Ang Li , Shruti Chaturvedi , Senthil Sukumar

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引用次数: 0

摘要

免疫介导的血栓性血小板减少性紫癜（iTTP）是一种罕见的血液系统疾病，由于治疗的进步，生存率提高。然而，心血管疾病的长期发病率和死亡率仍然很高。已建立的心血管风险计算方法，如2008年弗雷明汉心脏研究（FHS）全球心血管疾病（CVD）和美国心脏病学会/美国心脏协会（ACC/AHA）动脉粥样硬化性心血管疾病（ASCVD）风险估计值，可能不能充分解释iTTP幸存者心血管风险的升高和独特。目的评价ACC/AHA ASCVD和FHS全球CVD模型在预测iTTP幸存者主要不良心血管事件（mace）中的鉴别和校准。方法回顾性分析约翰霍普金斯大学1994-2024年间135例iTTP幸存者。包括心肌梗死、中风和心脏血运重建术在内的MACEs的存在是主要结局，并在临床缓解期间进行评估。采用c统计量评估模型的判别能力，采用Hosmer-Lemeshow检验和校准图评估模型的校准能力。计算敏感性、特异性、阳性预测值（PPV）和阴性预测值（NPV）。结果在中位随访3.8年期间，37.8%的队列发生了sace。ASCVD和FHS模型表现出较差的判别性（c-统计量分别为0.54和0.52）和较差的校准，观察到的MACE率超过预测概率（Hosmer-Lemeshow P < 0.05）。ASCVD模型敏感性为56.5%，特异性为49.4%，PPV为36.6%，NPV为64.9%；FHS模型敏感性为69.6%，特异性为39.3%，PPV为37.2%，NPV为67.9%。结论：标准心血管风险模型不能充分预测iTTP幸存者的MACE风险，强调需要定制工具，纳入iTTP特异性因素，以改善心血管风险分层和管理。

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Standard cardiovascular risk prediction scores underestimate risk in immune-mediated thrombotic thrombocytopenic purpura survivors

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 P < .05). The ASCVD model showed sensitivity of 56.5%, specificity of 49.4%, PPV of 36.6%, and NPV of 64.9%, while the FHS model showed sensitivity of 69.6%, specificity of 39.3%, PPV of 37.2%, and NPV of 67.9%.

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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