Thrombosis in the Philadelphia Chromosome-Negative Myeloproliferative Neoplasms.
Thrombosis in the Philadelphia Chromosome-Negative Myeloproliferative Neoplasms.
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DOI：
10.1007/978-3-030-20315-3_11
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发表时间：
2019-01-01
影响因子：
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通讯作者：
Rampal, Raajit K
中科院分区：
文献类型：
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作者：
Sankar, Kamya;Stein, Brady L;Rampal, Raajit K
骨髓增生性肿瘤（MPN）是克隆性干细胞源性疾病。本章重点介绍费城（Ph）染色体阴性经典MPN的亚类，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）。这些MPN与微血管和大血管血栓形成有关，可能发生在静脉和动脉循环中。已知红细胞增多、白细胞增多和JAK2V617F等位基因负荷增加是危险因素。在本章中，我们回顾了费城（Ph）染色体阴性的经典MPN的血栓和止血表现，包括临床表现，病理生理学，以及管理。
The myeloproliferative neoplasms (MPNs) are clonal stem cell-derived diseases. This chapter focuses on the subcategory of Philadelphia (Ph) chromosome-negative classical MPNs, polycythemia vera (PV), essential thrombocythemia (ET), primary myelofibrosis (PMF). These MPNs are associated with both microvascular and macrovascular thrombosis, which may occur in the venous and arterial circulation. Erythrocytosis, leukocytosis, and increased JAK2V617F allele burden are known to be risk factors. In this chapter, we review the thrombotic and hemostatic manifestations of the Philadelphia (Ph) chromosome-negative classical MPNs, including the clinical manifestations, the pathophysiology, as well as management.