Maleknia Mohsen, Shahrabi Saeid, Ghanavat Majid, Vosoughi Tina, Saki Najmaldin
Thalassemia & Hemoglobinopathy Research Center, Health Research Institute, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.
Student Research Committee, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.
Mol Biol Rep. 2020 Jun;47(6):4767-4778. doi: 10.1007/s11033-020-05536-x. Epub 2020 May 30.
Essential thrombocythemia (ET) is a classical myeloproliferative neoplasm that is susceptible to hypercoagulable state due to impaired hemostatic system, so that thrombotic complications are the leading cause of mortality in ET patients. The content used in this article has been obtained by the PubMed database and Google Scholar search engine from English-language articles (2000-2019) using the following keywords: "Essential thrombocythemia," "Thrombosis," "Risk factors" and "Hemostasis. In this neoplasm, the count and activity of cells such as platelets, leukocytes, endothelial cells, as well as erythrocytes are increased, which can increase the risk of thrombosis through rising intercellular interactions, expression of surface markers, and stimulation of platelet aggregation. In addition to these factors, genetic polymorphisms in hematopoietic stem cells (HSCs), including mutations in JAK2, CALR, MPL, or genetic abnormalities in other genes associated with the hemostatic system may be associated with increased risk of thrombotic events. Moreover, disruption of coagulant factors can pave the way for thrombogeneration. Therefore, the identification of markers related to cell activation, genetic abnormalities, or alternation in the coagulant system can be used together as diagnostic and prognostic markers for the occurrence of thrombosis among ET patients. Thus, because thrombotic complications are the main factors of mortality in ET patients, a hemostatic viewpoint and risk assessment of cellular, genetic, and coagulation factors can have prognostic value and contribute to the choice of effective treatment and prevention of thrombosis.
原发性血小板增多症(ET)是一种典型的骨髓增殖性肿瘤,由于止血系统受损,易发生高凝状态,因此血栓形成并发症是ET患者死亡的主要原因。本文所用内容通过PubMed数据库和谷歌学术搜索引擎,从2000 - 2019年的英文文章中获取,使用了以下关键词:“原发性血小板增多症”、“血栓形成”、“危险因素”和“止血”。在这种肿瘤中,血小板、白细胞、内皮细胞以及红细胞等细胞的数量和活性增加,可通过增强细胞间相互作用、表面标志物表达以及刺激血小板聚集来增加血栓形成风险。除这些因素外,造血干细胞(HSCs)中的基因多态性,包括JAK2、CALR、MPL突变,或与止血系统相关的其他基因的遗传异常,可能与血栓形成事件风险增加有关。此外,凝血因子的破坏可为血栓形成铺平道路。因此,与细胞活化、遗传异常或凝血系统改变相关的标志物的鉴定可共同用作ET患者血栓形成发生的诊断和预后标志物。因此,由于血栓形成并发症是ET患者死亡的主要因素,从止血角度以及对细胞、遗传和凝血因子的风险评估可具有预后价值,并有助于选择有效的治疗方法和预防血栓形成。