a Department of Hematology , Johns Hopkins School of Medicine , Baltimore , MD , USA.
Expert Rev Hematol. 2019 Mar;12(3):173-182. doi: 10.1080/17474086.2019.1583554. Epub 2019 Mar 8.
Venous thromboembolism (VTE) is a common comorbid condition found in sickle cell disease (SCD) and is associated with increased mortality for adults with SCD. The pathophysiology that leads to the thrombophilic state in SCD has been previously reviewed; however, evidence-based guidelines to aid in diagnosis, prevention, and management of VTE are lacking. Areas covered: This review article will cover the pathophysiology underlying the hypercoagulable state, the epidemiology of VTE, and management strategies of VTE in SCD. Expert opinion: Providers should have a high suspicion for diagnosing VTE to help reduce morbidity and mortality in the SCD population. Unlike other thrombophilias, the risk of life-threatening anemia while being treated with anticoagulation is compounded with the potential complications surrounding red blood cell transfusions in this population (i.e. alloimmunization, hyperhemolysis) and this provides another complexity to managing VTE in this population. Clinical trials evaluating the risk and benefit of treatment and treatment duration are needed.
静脉血栓栓塞症（VTE）是镰状细胞病（SCD）中常见的合并症，与 SCD 成年患者的死亡率增加有关。导致 SCD 中血栓形成状态的病理生理学已被先前综述；然而，缺乏有助于 VTE 的诊断、预防和管理的循证指南。涵盖领域：这篇综述文章将涵盖导致高凝状态的病理生理学、VTE 的流行病学以及 SCD 中 VTE 的管理策略。专家意见：提供者应高度怀疑诊断 VTE，以帮助降低 SCD 人群的发病率和死亡率。与其他血栓形成倾向不同，在接受抗凝治疗时，因危及生命的贫血而产生的风险与该人群中红细胞输血相关的潜在并发症（即同种免疫、高红细胞溶解）相结合，这为管理该人群中的 VTE 增加了另一个复杂性。需要评估治疗和治疗持续时间风险和益处的临床试验。
