Prajs Iwona, Kuliczkowski Kazimierz
Department and Clinic of Hematology, Blood Neoplasms, and Bone Marrow Transplantation, Wroclaw Medical University, Poland.
Adv Clin Exp Med. 2017 Jan-Feb;26(1):115-121. doi: 10.17219/acem/68578.
Thrombotembolic complications are the leading cause of mortality in essential thrombocythemia (ET), but the definition of thrombotic risk remains far from clear.
The aim of this study was to evaluate the prognostic markers for thrombosis to identify ET patients at risk.
Forty-five consecutive patients with ET were studied. This group was divided into two subgroups ET patients with (A) and without (B) history of thrombosis. Each patient has been tested for complete blood count, fibrinogen, factor VIII, D-dimer, protein C, APCR, TAT and F1+2. JAK2 mutation was assessed by RT-PCR. Factor V Leiden and prothrombin genes mutations were screened by DNA sequencing.
The median age of ET patients was 62.0 years. JAK2 mutation was found in 24 patients, 21 of them had a history of thrombotic events, and 17/21 were JAK2 positive. Compared to controls, ET patients had a significantly higher WBC and PLT counts, and higher mean platelet volume (MPV), but not Hgb level or RBC count. In ET subgroup A, apart from changes seen in the whole ET, the Hgb level, RBC count, and Hct were also significantly elevated. Interestingly, the MPV was significantly larger in subgroup A, but not in B. Fibrinogen and D-dimers levels were significantly higher in ET group than in controls, but not F1+F2 or TAT. The results of hemostatic tests did not markedly differ between subgroups A and B. APCR was found in 5/45 patients with ET, and 2 out of 5 had a factor V Leiden heterozygous mutation. No prothrombin gene mutation was observed.
Our results suggest that MPV can serve as a simple test for assessing the hypercoagulable state in ET patients. It has been confirmed that JAK2 mutation and leukocytosis are independent predictors for thrombotic events in ET patients.
血栓栓塞并发症是原发性血小板增多症(ET)患者死亡的主要原因,但血栓形成风险的定义仍远未明确。
本研究旨在评估血栓形成的预后标志物,以识别有风险的ET患者。
对45例连续性ET患者进行研究。该组患者被分为两个亚组:有(A)和无(B)血栓形成病史的ET患者。对每位患者进行了全血细胞计数、纤维蛋白原、凝血因子VIII、D-二聚体、蛋白C、活化蛋白C抵抗(APCR)、凝血酶-抗凝血酶复合物(TAT)和F1+2检测。通过逆转录聚合酶链反应(RT-PCR)评估JAK2突变。通过DNA测序筛查凝血因子V莱顿突变和凝血酶原基因突变。
ET患者的中位年龄为62.0岁。24例患者检测到JAK2突变,其中21例有血栓形成事件史,且17/21例JAK2呈阳性。与对照组相比,ET患者的白细胞(WBC)和血小板(PLT)计数显著更高,平均血小板体积(MPV)也更高,但血红蛋白(Hgb)水平或红细胞(RBC)计数无差异。在ET亚组A中,除了整个ET组出现的变化外,Hgb水平、RBC计数和血细胞比容(Hct)也显著升高。有趣的是,亚组A的MPV显著更大,而亚组B则不然。ET组的纤维蛋白原和D-二聚体水平显著高于对照组,但F1+F2或TAT无差异。亚组A和B之间的止血检测结果无明显差异。45例ET患者中有5例检测到APCR,其中5例中有2例有凝血因子V莱顿杂合突变。未观察到凝血酶原基因突变。
我们的结果表明,MPV可作为评估ET患者高凝状态的一项简单检测指标。已证实JAK2突变和白细胞增多是ET患者血栓形成事件的独立预测因素。