Kaifie A, Kirschner M, Wolf D, Maintz C, Hänel M, Gattermann N, Gökkurt E, Platzbecker U, Hollburg W, Göthert J R, Parmentier S, Lang F, Hansen R, Isfort S, Schmitt K, Jost E, Serve H, Ehninger G, Berdel W E, Brümmendorf T H, Koschmieder S
Department of Hematology, Oncology, Hemostaseology, and Stem Cell Transplantation, Faculty of Medicine, RWTH Aachen University, Pauwelsstr. 30, 52074, Aachen, Germany.
Internal Medicine 3, Oncology, Hematology and Rheumatology, University Clinic Bonn (UKB), Bonn, Germany.
J Hematol Oncol. 2016 Mar 5;9:18. doi: 10.1186/s13045-016-0242-9.
Patients with Ph-negative myeloproliferative neoplasms (MPN), such as polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF), are at increased risk for thrombosis/thromboembolism and major bleeding. Due to the morbidity and mortality of these events, antiplatelet and/or anticoagulant agents are commonly employed as primary and/or secondary prophylaxis. On the other hand, disease-related bleeding complications (i.e., from esophageal varices) are common in patients with MPN. This analysis was performed to define the frequency of such events, identify risk factors, and assess antiplatelet/anticoagulant therapy in a cohort of patients with MPN.
The MPN registry of the Study Alliance Leukemia is a non-interventional prospective study including adult patients with an MPN according to WHO criteria (2008). For statistical analysis, descriptive methods and tests for significant differences as well as contingency tables were used to identify the odds of potential risk factors for vascular events.
MPN subgroups significantly differed in sex distribution, age at diagnosis, blood counts, LDH levels, JAK2V617F positivity, and spleen size (length). While most thromboembolic events occurred around the time of MPN diagnosis, one third of these events occurred after that date. Splanchnic vein thrombosis was most frequent in post-PV-MF and MPN-U patients. The chance of developing a thromboembolic event was significantly elevated if patients suffered from post-PV-MF (OR 3.43; 95% CI = 1.39-8.48) and splenomegaly (OR 1.76; 95% CI = 1.15-2.71). Significant odds for major bleeding were previous thromboembolic events (OR = 2.71; 95% CI = 1.36-5.40), splenomegaly (OR = 2.22; 95% CI 1.01-4.89), and the administration of heparin (OR = 5.64; 95% CI = 1.84-17.34). Major bleeding episodes were significantly less frequent in ET patients compared to other MPN subgroups.
Together, this report on an unselected "real-world" cohort of German MPN patients reveals important data on the prevalence, diagnosis, and treatment of thromboembolic and major bleeding complications of MPN.
Ph阴性骨髓增殖性肿瘤(MPN)患者,如真性红细胞增多症(PV)、原发性血小板增多症(ET)和原发性骨髓纤维化(PMF),发生血栓形成/血栓栓塞和严重出血的风险增加。由于这些事件的发病率和死亡率,抗血小板和/或抗凝药物通常被用作一级和/或二级预防。另一方面,MPN患者中与疾病相关的出血并发症(即来自食管静脉曲张)很常见。进行这项分析是为了确定此类事件的发生频率,识别风险因素,并评估一组MPN患者的抗血小板/抗凝治疗。
白血病研究联盟的MPN注册研究是一项非干预性前瞻性研究,纳入了符合WHO标准(2008年)的成年MPN患者。为了进行统计分析,使用描述性方法、显著性差异检验以及列联表来确定血管事件潜在风险因素的几率。
MPN亚组在性别分布、诊断时年龄、血细胞计数、乳酸脱氢酶水平、JAK2V617F阳性率和脾脏大小(长度)方面存在显著差异。虽然大多数血栓栓塞事件发生在MPN诊断前后,但其中三分之一的事件发生在诊断日期之后。内脏静脉血栓形成在PV后骨髓纤维化和MPN未分类患者中最为常见。如果患者患有PV后骨髓纤维化(比值比3.43;95%置信区间=1.39 - 8.48)和脾肿大(比值比1.76;95%置信区间=1.15 - 2.71),发生血栓栓塞事件的几率会显著升高。严重出血的显著几率因素包括既往血栓栓塞事件(比值比=2.71;95%置信区间=1.36 - 5.40)、脾肿大(比值比=2.22;95%置信区间1.01 - 4.89)和肝素的使用(比值比=5.64;95%置信区间=1.84 - 17.34)。与其他MPN亚组相比,ET患者的严重出血事件明显较少。
这份关于德国MPN患者未经过选择的“真实世界”队列报告,共同揭示了MPN血栓栓塞和严重出血并发症的患病率、诊断和治疗的重要数据。