Oncologic Center, Department of Internal Medicine III with Haematology, Medical Oncology, Haemostaseology, Infectiology and Rheumatology, Paracelsus Medical University, 5020 Salzburg, Austria.
Cancer Cluster Salzburg, 5020 Salzburg, Austria.
Int J Mol Sci. 2021 Sep 3;22(17):9555. doi: 10.3390/ijms22179555.
Philadelphia chromosome negative myeloproliferative neoplasms (MPN) are composed of polycythemia vera (PV), essential thrombocytosis (ET), and primary myelofibrosis (PMF). The clinical picture is determined by constitutional symptoms and complications, including arterial and venous thromboembolic or hemorrhagic events. MPNs are characterized by mutations in , , or , with additional mutations leading to an expansion of myeloid cell lineages and, in PMF, to marrow fibrosis and cytopenias. Chronic inflammation impacting the initiation and expansion of disease in a major way has been described. Neutrophilic granulocytes play a major role in the pathogenesis of thromboembolic events via the secretion of inflammatory markers, as well as via interaction with thrombocytes and the endothelium. In this review, we discuss the molecular biology underlying myeloproliferative neoplasms and point out the central role of leukocytosis and, specifically, neutrophilic granulocytes in this group of disorders.
费城染色体阴性骨髓增殖性肿瘤（MPN）包括真性红细胞增多症（PV）、原发性血小板增多症（ET）和原发性骨髓纤维化（PMF）。临床表现由全身症状和并发症决定，包括动静脉血栓栓塞或出血事件。MPN 的特征是存在 、 或 基因突变，此外还有导致髓系细胞系扩增的其他突变，在 PMF 中导致骨髓纤维化和细胞减少。已经描述了慢性炎症以主要方式影响疾病的发生和发展。中性粒细胞通过分泌炎症标志物以及与血小板和内皮细胞相互作用，在血栓栓塞事件的发病机制中起主要作用。在这篇综述中，我们讨论了骨髓增殖性肿瘤的分子生物学，并指出白细胞增多症，特别是中性粒细胞在这组疾病中的核心作用。
