Sukumar Senthil, Mazepa Marshall A, Chaturvedi Shruti
Division of Hematology/Oncology, Department of Medicine, Baylor College of Medicine, Houston, TX 77098, USA.
Division of Hematology, Oncology, and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, MN 55455, USA.
J Clin Med. 2023 Sep 14;12(18):5961. doi: 10.3390/jcm12185961.
Advances in the management of immune thrombotic thrombocytopenic purpura (iTTP) have dramatically improved outcomes of acute TTP episodes, and TTP is now treated as a chronic, relapsing disorder. It is now recognized that iTTP survivors are at high risk for vascular disease, with stroke and myocardial infarction occurring at younger ages than in the general population, and cardiovascular disease is the leading cause of premature death in this population. iTTP appears to have a phenotype of accelerated vascular aging with a particular predilection for cerebral circulation, and stroke is much more common than myocardial infarction. In addition to traditional cardiovascular risk factors, low ADAMTS13 activity during clinical remission may be a risk factor for some of these outcomes, such as stroke. Recent studies also suggest that Black patients, who are disproportionately affected by iTTP in the United States, are at higher risk of adverse cardiovascular outcomes, likely due to multifactorial reasons. Additional research is required to establish the risk factors and mechanisms underlying these complications in order to institute optimal screening strategies and identify interventions to improve outcomes.
免疫性血栓性血小板减少性紫癜（iTTP）管理方面的进展显著改善了急性TTP发作的治疗结果，现在TTP被视为一种慢性复发性疾病。目前人们认识到，iTTP幸存者患血管疾病的风险很高，中风和心肌梗死的发病年龄比普通人群更年轻，心血管疾病是该人群过早死亡的主要原因。iTTP似乎具有血管加速老化的表型，特别倾向于脑循环，中风比心肌梗死更为常见。除了传统的心血管危险因素外，临床缓解期ADAMTS13活性低可能是这些结局（如中风）的危险因素之一。最近的研究还表明，在美国受iTTP影响尤为严重的黑人患者，发生不良心血管结局的风险更高，这可能是多种因素导致的。需要进一步研究以确定这些并发症的危险因素和潜在机制，从而制定最佳筛查策略并确定改善结局的干预措施。
