Department of Haematology, University Hospital Galway, Galway, Republic of Ireland.
Department of Haematology, The Royal London Hospital, London, UK.
Br J Haematol. 2021 Sep;194(5):822-834. doi: 10.1111/bjh.17390. Epub 2021 Apr 6.
Immune thrombocytopenia (ITP) is an autoimmune disorder in which a combination of defective platelet production and enhanced clearance leads to thrombocytopenia. The primary aim for therapy in patients with this condition is the prevention of bleeding. However, more recently, increased rates of venous and arterial thrombotic events have been reported in ITP, even in the context of marked thrombocytopenia. In this review we discuss the epidemiology, aetiology and management of thrombotic events in these patients. We consider the impact of ITP therapies on the increased thrombotic risk, in particular the use of thrombopoietin-receptor agonists (TPO-RAs), as well as factors inherent to ITP itself. We also discuss the limited evidence available to guide clinicians in the treatment of these complex cases.
免疫性血小板减少症 (ITP) 是一种自身免疫性疾病，其特征是血小板生成缺陷和清除增强导致血小板减少。此类患者的治疗主要目标是预防出血。然而，最近有报道称，即使在血小板显著减少的情况下，ITP 患者也会出现静脉和动脉血栓栓塞事件的发生率增加。在这篇综述中，我们讨论了这些患者血栓形成事件的流行病学、病因学和管理。我们考虑了 ITP 治疗对增加的血栓形成风险的影响，特别是使用血小板生成素受体激动剂 (TPO-RAs)，以及 ITP 本身固有的因素。我们还讨论了指导临床医生治疗这些复杂病例的有限证据。
