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巨核细胞在骨髓纤维化中的作用。

The Role of Megakaryocytes in Myelofibrosis.

机构信息

Division of Experimental Hematology, St. Jude Children's Research Hospital, 262 Danny Thomas Place, MC341, Memphis, TN 38105, USA. Electronic address: https://twitter.com/melo_cardenas1.

Department of Biomedical and Neuromotor Sciences, University of Bologna, Via Irnerio, 48 - I 40126, Bologna, Italy.

出版信息

Hematol Oncol Clin North Am. 2021 Apr;35(2):191-203. doi: 10.1016/j.hoc.2020.11.004. Epub 2021 Jan 11.

Abstract

Megakaryocytes give rise to platelets, which have a wide variety of functions in coagulation, immune response, inflammation, and tissue repair. Dysregulation of megakaryocytes is a key feature of in the myeloproliferative neoplasms, especially myelofibrosis. Megakaryocytes are among the main drivers of myelofibrosis by promoting myeloproliferation and bone marrow fibrosis. In vivo targeting of megakaryocytes by genetic and pharmacologic approaches ameliorates the disease, underscoring the important role of megakaryocytes in myeloproliferative neoplasms. Here we review the current knowledge of the function of megakaryocytes in the JAK2, CALR, and MPL-mutant myeloproliferative neoplasms.

摘要

巨核细胞生成血小板,血小板在凝血、免疫反应、炎症和组织修复中具有多种功能。巨核细胞的失调是骨髓增生性肿瘤的一个关键特征,尤其是骨髓纤维化。巨核细胞通过促进骨髓增生和骨髓纤维化成为骨髓纤维化的主要驱动因素之一。通过遗传和药理学方法在体内靶向巨核细胞可改善疾病,这突显了巨核细胞在骨髓增生性肿瘤中的重要作用。在这里,我们回顾了巨核细胞在 JAK2、CALR 和 MPL 突变型骨髓增生性肿瘤中的功能的现有知识。

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