骨髓增殖性肿瘤血栓形成的研究进展

Advances in mechanisms and treatment of thrombocytopenia in myelodysplastic syndromes

经典的Ph－慢性骨髓增殖性肿瘤（MPN），包括真性红细胞增多症（PV）、原发性血小板增多症（ET）、原发性骨髓纤维化（PMF），是一组起源于造血干细胞的恶性骨髓增殖性疾病。其中，动静脉血栓形成是MPN的主要并发症与患者致残、致死的重要原因之一。目前研究结果表明，高龄（年龄〉60岁），既往血栓病史，均为MPN患者血栓形成最重要的危险因素。白细胞计数增高与白细胞激活为血栓形成的危险因素。同时有研究者指出，Janus激酶（JAK）2 V617F基因、钙网蛋白（CALR）基因、MPL等基因突变，亦影响MPN患者的血栓形成。MPN患者在接受治疗前需评估血栓形成的风险，针对患者血栓形成的风险，有目的性地采用不同的治疗方法。笔者拟就高龄（年龄〉60岁），既往血栓病史，心血管疾病危险因素，JAK2、MPL、CALR等基因突变，白细胞计数增高等方面对MPN血栓形成的影响，以及MPN的血栓形成风险评估与治疗等方面的研究进展进行综述。

Myelodysplastic syndromes （MDS） are a group of bone marrow failure diseases. Thrombocytopenia in MDS is a major cause of high mortality for MDS patients. Incidence of thrombocytopenia in MDS is 40%-65% and mechanisms of thrombocytopenia in MDS are complicated. Thrombocytopenia in MDS is an independent factor predicting poor prognosis contributing to hemorrhagic complications, low survival rate and increasing risk of conversion to acute myeloblastic leukemia （AML）. Platelet transfusion is still the standard treatment option thromboeytopenia in MDS. The new generation thrombopoietin （TPO） receptor agonists have shown curative effect on thrombocytopenia in MDS patients in preliminary clinical trials. In order to further clarify the characteristic of thrombocytopenia in MDS, this article focuses literatures on mechanisms, treatment progress and prognosis of thrombocytopenia in MDS.