Department of Hematology and Oncology, Charite Berlin, Berlin, Hindenburgdamm 30, 12200, Berlin, Germany,
Ann Hematol. 2014 Dec;93(12):1953-63. doi: 10.1007/s00277-014-2224-8. Epub 2014 Oct 14.
Patients with Philadelphia chromosome-negative myeloproliferative neoplasms (MPN) like polycythemia vera and essential thrombocythemia are at increased risk of arterial and venous thrombosis. Strategies of prevention may consist of platelet aggregation inhibitors and/or cytoreductive agents depending on the underlying disease and the individual risk. Clinical evidence for management of acute venous thromboembolic events in MPN patients is limited. Modality and duration of therapeutic anticoagulation after venous thrombosis has to be evaluated critically with special regard to the increased risk for spontaneous bleeding events associated with the underlying diseases. Both for therapy of the acute event and for secondary prophylaxis, low-molecular-weight heparins should preferentially be used. A prolongation of the therapeutic anticoagulation beyond the usual 3 to 6 months can only be recommended in high-risk settings and after careful evaluation of potential risks and benefits for the individual patient. New direct oral anticoagulants (NOAC) should not preferentially be used due to lack of clinical experience in patients with MPN and potential drug interactions (e.g. with JAK inhibitors). Consequent treatment of the underlying myeloproliferative disease and periodical evaluation of the response to therapy is crucial for optimal secondary prophylaxis of thromboembolic events in those patients.
费城染色体阴性骨髓增殖性肿瘤(MPN)患者,如真性红细胞增多症和原发性血小板增多症,发生动脉和静脉血栓的风险增加。预防策略可能包括血小板聚集抑制剂和/或细胞减灭剂,具体取决于基础疾病和个体风险。MPN 患者急性静脉血栓栓塞事件管理的临床证据有限。静脉血栓形成后治疗性抗凝的方式和持续时间必须根据基础疾病相关自发性出血事件风险增加进行严格评估。对于急性事件的治疗和二级预防,应优先使用低分子量肝素。只有在高危情况下,并仔细评估个体患者的潜在风险和获益后,才能延长治疗性抗凝超过通常的 3 至 6 个月。由于缺乏 MPN 患者的临床经验和潜在药物相互作用(如与 JAK 抑制剂),新型口服抗凝药物(NOAC)不应优先使用。对于那些患者,治疗基础骨髓增殖性疾病和定期评估治疗反应对于血栓栓塞事件的最佳二级预防至关重要。