Asakura Hidesaku
Department of Internal Medicine (III), Kanazawa University School of Medicine.
Rinsho Ketsueki. 2018;59(8):1034-1041. doi: 10.11406/rinketsu.59.1034.
Among all types of myeloproliferative neoplasms (MPNs), polycythemia vera (PV) and essential thrombocythemia (ET) require careful management of both thrombosis and hemostasis. One recent concern associated with MPNs is the JAK2 mutation (V617F). This mutation is essential for MPN pathology, but it has also garnered attention for its association with thrombosis. Several studies have reported the mechanisms underlying the onset of thrombosis in both PV and ET and have also discussed the association between JAK2 mutations and thrombotic tendencies. Arterial thrombosis is a common clinical symptom that is associated with the diagnosis and course of both PV and ET. Particularly, cerebral infarction has been identified as the leading cause of death in patients with untreated PV. Notably, PV and ET are also associated with a high incidence of venous thromboembolism (VTE). The occurrence of this type of thrombosis at atypical sites, such as cerebral venous sinus thrombosis and splanchnic vein thrombosis (SVT), is not uncommon. Generally, patients with PV and ET have good life expectancy; their treatment essentially focuses on dealing with thrombosis and bleeding. Phlebotomy may be used for treating patients with PV; however, low-dose aspirin is used to prevent the onset of arterial thrombosis. For patients with a history of VTE, oral anticoagulants are commonly prescribed to prevent recurrence.
在所有类型的骨髓增殖性肿瘤(MPN)中,真性红细胞增多症(PV)和原发性血小板增多症(ET)都需要对血栓形成和止血进行仔细管理。最近与MPN相关的一个问题是JAK2突变(V617F)。这种突变对于MPN的病理过程至关重要,但它也因其与血栓形成的关联而受到关注。几项研究报告了PV和ET中血栓形成的发病机制,并且还讨论了JAK2突变与血栓形成倾向之间的关联。动脉血栓形成是一种常见的临床症状,与PV和ET的诊断及病程相关。特别是,脑梗死已被确定为未经治疗的PV患者的主要死因。值得注意的是,PV和ET还与静脉血栓栓塞(VTE)的高发病率相关。这种类型的血栓形成发生在非典型部位,如脑静脉窦血栓形成和内脏静脉血栓形成(SVT),并不罕见。一般来说,PV和ET患者的预期寿命良好;他们的治疗主要集中在处理血栓形成和出血。放血疗法可用于治疗PV患者;然而,低剂量阿司匹林用于预防动脉血栓形成。对于有VTE病史的患者,通常会开口服抗凝剂以预防复发。