静脉和动脉血栓栓塞是骨髓增生性肿瘤（MPN）大的并发症，包括红细胞增多症（PV）、 essential thrombocythemia（ET）和原发性骨髓纤维化（PMF）。全球止血试验，包括凝血酶生成试验（TGA）、旋转血栓弹性图法（ROTEM）和血栓弹性图（TEG），已被提议作为生物标志物，评估MPN中的高凝状态和血栓风险分层。我们对TGA、ROTEM和TEG的参数及其与MPN中的血栓事件和治疗策略的相关性进行了系统的文献综述。共纳入32项研究（全部为横断面研究），共有1,062个对照组和1,608个MPN患者。在13项报告动脉或静脉血栓形成的研究中，总的血栓形成率为13.8%，其中报告了6例腹部血栓形成。在27项TGA研究中，实验室试验中使用的血浆制备和触发试剂存在显著的异质性。所有MPN队列与对照组相比峰高有增加的趋势，ET患者与对照组之间的内源性凝血酶原潜能（ETP）较高。PV和PMF患者与对照组之间，ETP总体趋势向较低方向。在JAK2阳性与JAK2阴性MPN，先前血栓事件史与否以及不同治疗策略之间，ETP没有实质性差异。在三个ROTEM研究中，所有MPNs与对照组相比最大凝块硬度更高，凝块形成时间更短的趋势。三项TEG研究结果各不相同。我们得出结论，全球止血试验参数预测MPN患者高凝状态事件的能力不一致和不确定。需要进一步的前瞻性长期研究来验证这些生物标志物工具，以便血栓形成潜力可以作为这些研究的主要终点。Thieme。版权所有。

Venous and arterial thromboembolism are major complications of myeloproliferative neoplasms (MPNs), comprising polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF). Global hemostasis assays, including thrombin generation assay (TGA), rotational thromboelastometry (ROTEM), and thromboelastography (TEG), have been proposed as biomarkers to assess the hypercoagulability and thrombotic risk stratification in MPNs. We performed a systematic literature review on the parameters of TGA, ROTEM, and TEG and their association with thrombotic events and treatment strategies in MPNs. Thirty-two studies (all cross-sectional) were included, which collectively enrolled 1,062 controls and 1,608 MPN patients. Among the 13 studies that reported arterial or venous thrombosis, the overall thrombosis rate was 13.8% with 6 splanchnic thromboses reported. Out of the 27 TGA studies, there was substantial heterogeneity in plasma preparation and trigger reagents employed in laboratory assays. There was a trend toward increased peak height among all MPN cohorts versus controls and higher endogenous thrombin potential (ETP) between ET patients versus controls. There was an overall trend toward lower ETP between PV and PMF patients versus. controls. There were no substantial differences in ETP between JAK2-positive versus JAK2-negative MPNs, prior history versus negative history of thrombotic events, and among different treatment strategies. Of the three ROTEM studies, there was a trend toward higher maximum clot firmness and shorter clot formation times for all MPNs versus controls. The three TEG studies had mixed results. We conclude that the ability of parameters from global hemostasis assays to predict for hypercoagulability events in MPN patients is inconsistent and inconclusive. Further prospective longitudinal studies are needed to validate these biomarker tools so that thrombotic potential could be utilized as a primary endpoint of such studies.Thieme. All rights reserved.