Thrombotic and hemorrhagic complications are prevalent in patients with essential thrombocythemia, polycythemia vera, and myelofibrosis. Given the impact on morbidity and mortality, reducing the risk of thrombosis and/or hemorrhage is a major therapeutic goal. Historically, patients have been risk stratified on the basis of traditional factors, such as advanced age and thrombosis history. However, multiple factors contribute to the thrombotic tendency, including gender, mutational profile, inflammatory stress, and abnormal cell adhesion. Management includes cardiovascular risk reduction and use of antiplatelet therapy, depending on myeloproliferative neoplasm subtype and mutational status. Anticoagulation is a mainstay of therapy for those with venous thrombosis, but practice patterns remain heterogeneous. Cytoreduction is indicated for higher-risk patients, but efficacy may depend on the involved vascular bed. Management of special situations, such as unusual site thrombosis, bleeding, the perioperative period, and pregnancy, are especially challenging. In this article, risk factors and treatment strategies for myeloproliferative neoplasm thrombosis and bleeding, including special situations, are reviewed. Insights gained from recent studies may lead to the development of a more precise risk classification and tailored therapy.

中文翻译：

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从 Budd-Chiari 综合征到获得性 von Willebrand 综合征：骨髓增殖性肿瘤中的血栓形成和出血并发症

血栓性和出血性并发症在原发性血小板增多症、真性红细胞增多症和骨髓纤维化患者中普遍存在。鉴于对发病率和死亡率的影响，降低血栓形成和/或出血的风险是主要的治疗目标。从历史上看，患者已根据传统因素进行风险分层，例如高龄和血栓形成史。然而，多种因素导致血栓形成趋势，包括性别、突变特征、炎症应激和异常细胞粘附。管理包括降低心血管风险和使用抗血小板治疗，这取决于骨髓增殖性肿瘤亚型和突变状态。抗凝是静脉血栓形成患者的主要治疗方法，但实践模式仍然存在差异。细胞减灭术适用于高危患者，但疗效可能取决于所涉及的血管床。特殊情况的处理，如异常部位血栓形成、出血、围手术期和妊娠，尤其具有挑战性。本文综述了骨髓增殖性肿瘤血栓形成和出血的危险因素和治疗策略，包括特殊情况。从最近的研究中获得的见解可能会导致更精确的风险分类和定制治疗的发展。包括特殊情况，进行审查。从最近的研究中获得的见解可能会导致更精确的风险分类和定制治疗的发展。包括特殊情况，进行审查。从最近的研究中获得的见解可能会导致更精确的风险分类和定制治疗的发展。