Department of Laboratory Diagnostics, University Hospital Centre Zagreb, Zagreb, Croatia.
Department of Hematology, University Hospital Centre Zagreb, Zagreb, Croatia; School of Medicine, University of Zagreb, Zagreb, Croatia.
Clin Lymphoma Myeloma Leuk. 2019 Jan;19(1):53-63. doi: 10.1016/j.clml.2018.08.020. Epub 2018 Sep 10.
Thrombosis is the most common complication in Philadelphia chromosome negative (Ph-) myeloproliferative neoplasms patients.
In a cohort of 258 Ph- myeloproliferative neoplasm patients, the difference between patients with and without thrombosis was analyzed according to genetic thrombophilia factors, JAK2 V617F status and burden allele, blood count, cardiovascular risk factors and age. Patients were also divided in polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) subgroups as well as by the type of thrombosis.
Analysis of cardiovascular risk factors regarding arterial thrombosis showed that PV patients with thrombosis had higher incidence of diabetes (P = .030), ET patients more often had hypertension (P = .003) and hyperlipidemia (P = .005), while PMF patients had hyperlipidemia (P = .046) and at least one cardiovascular risk factor (P = .044). Moreover, leukocytes > 18 × 10/L and V617F burden allele > 25.7% were statistically significantly different in PV patients (P = .019 and borderline significant at P = .055, respectively), while in ET patients leukocytes > 9.2 × 10/L (P < .001) and age at diagnosis of > 55 years were statistically significantly different (P = .002). PMF patients with V617F burden allele ≤ 34.8% were more prone to thrombosis (P = .032). When comparing patients with and without venous thrombosis, cutoff value of V617F burden allele > 90.4% was significant for PV patients with thrombosis (P = .036), as was > 56.7% for PMF patients with thrombosis (P = .046). Platelets ≤ 536 × 10/L and age at diagnosis > 54 years showed statistically significant difference for ET patients with thrombosis (P = .015 and P = .041, respectively).
On the basis of our results, a new scoring system for thrombosis risk in PV could be made, while PMF prognostic model may be expanded for better recognition of potential thrombotic risk factors.
血栓形成是费城染色体阴性(Ph-)骨髓增殖性肿瘤患者最常见的并发症。
在 258 例 Ph-骨髓增殖性肿瘤患者的队列中,根据遗传血栓形成因素、JAK2 V617F 状态和负荷等位基因、血细胞计数、心血管危险因素和年龄,分析了有血栓形成和无血栓形成患者之间的差异。患者还分为真性红细胞增多症(PV)、原发性血小板增多症(ET)和原发性骨髓纤维化(PMF)亚组,并根据血栓形成的类型进行分组。
关于动脉血栓形成的心血管危险因素分析显示,PV 合并血栓形成的患者中糖尿病的发生率更高(P =.030),ET 患者更常合并高血压(P =.003)和高脂血症(P =.005),而 PMF 患者更常合并高脂血症(P =.046)和至少一种心血管危险因素(P =.044)。此外,PV 患者白细胞计数 > 18×10/L 和 V617F 负荷等位基因 > 25.7%有统计学差异(P =.019 和边缘显著,P =.055),而 ET 患者白细胞计数 > 9.2×10/L(P <.001)和诊断时年龄 > 55 岁有统计学差异(P =.002)。PMF 患者 V617F 负荷等位基因 ≤ 34.8%更易发生血栓形成(P =.032)。比较有静脉血栓形成和无静脉血栓形成的患者,PV 患者血栓形成时 V617F 负荷等位基因 > 90.4%的截断值有统计学意义(P =.036),PMF 患者血栓形成时 > 56.7%的截断值有统计学意义(P =.046)。ET 患者血栓形成时血小板计数 ≤ 536×10/L 和诊断时年龄 > 54 岁有统计学差异(P =.015 和 P =.041)。
基于我们的结果,可以为 PV 制定新的血栓形成风险评分系统,同时可以扩展 PMF 预后模型以更好地识别潜在的血栓形成危险因素。