Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
Division of Hematology, Department of Medicine, The Ohio State University College of Medicine, Columbus, Ohio, USA.
Am J Hematol. 2021 Dec 1;96(12):1587-1594. doi: 10.1002/ajh.26341. Epub 2021 Sep 15.
Cardiovascular disease is a leading cause of death in survivors of immune-mediated thrombotic thrombocytopenic purpura (iTTP), but the epidemiology of major adverse cardiovascular events (MACE) in iTTP survivors is unknown. We evaluated the prevalence and risk factors for MACE, defined as the composite of non-fatal or fatal myocardial infarction (MI), stroke, and cardiac revascularization, during clinical remission in two large iTTP cohorts (Johns Hopkins University and Ohio State University). Of 181 patients followed for ≥ 3 months after recovery from acute iTTP, 28.6% had a MACE event over a median follow up of 7.6 years. Stroke was the most common type of MACE (18.2%), followed by non-fatal MI (6.6%), cardiac revascularization (4.9%) and fatal MI (0.6%). Compared to the general United States population, iTTP survivors were younger at first stroke in remission (males [56.5 years vs. 68.6 years, p = 0.031], females [49.7 years vs. 72.9 years, p < 0.001]) or MI in remission (males [56.5 years vs. 65.6 years, p < 0.001] and females [53.1 years vs. 72.0 years, p < 0.001]). Age (HR 1.03 [95% CI 1.002-1.054]), race (Black/Other vs. White) (HR 2.32 [95% CI 1.12-4.82]), and diabetes mellitus (HR 2.37 [95% CI 1.09-0.03]) were associated with MACE in a Cox regression model also adjusted for sex, hypertension, obesity, hyperlipidemia, chronic kidney disease, atrial fibrillation, autoimmune disease, and relapsing iTTP. Remission ADAMTS13 activity was not significantly associated with MACE. In conclusion, iTTP survivors experience high rates of MACE and may benefit from aggressively screening for and managing cardiovascular risk factors.
心血管疾病是免疫介导性血栓性血小板减少性紫癜（iTTP）幸存者的主要死亡原因，但 iTTP 幸存者发生主要不良心血管事件（MACE）的流行病学情况尚不清楚。我们评估了在两个大型 iTTP 队列（约翰霍普金斯大学和俄亥俄州立大学）的临床缓解期内，MACE（非致死性或致死性心肌梗死（MI）、中风和心脏血运重建的复合事件）的发生率和危险因素。在 181 例急性 iTTP 恢复后至少随访 3 个月的患者中，中位随访 7.6 年后有 28.6%发生了 MACE 事件。中风是最常见的 MACE 类型（18.2%），其次是非致死性 MI（6.6%）、心脏血运重建（4.9%）和致死性 MI（0.6%）。与美国普通人群相比，iTTP 幸存者在缓解期的首次中风（男性[56.5 岁比 68.6 岁，p=0.031]，女性[49.7 岁比 72.9 岁，p<0.001]）或缓解期的 MI（男性[56.5 岁比 65.6 岁，p<0.001]和女性[53.1 岁比 72.0 岁，p<0.001]）的年龄较小。年龄（HR 1.03 [95% CI 1.002-1.054]）、种族（黑人/其他族裔比白人）（HR 2.32 [95% CI 1.12-4.82]）和糖尿病（HR 2.37 [95% CI 1.09-0.03]）与 Cox 回归模型中的 MACE 相关，该模型还调整了性别、高血压、肥胖、血脂异常、慢性肾脏病、心房颤动、自身免疫性疾病和复发性 iTTP。缓解期 ADAMTS13 活性与 MACE 无显著相关性。总之，iTTP 幸存者发生 MACE 的比率较高，可能需要积极筛查和管理心血管危险因素。
