Essential thrombocythemia vs. early/prefibrotic myelofibrosis: Why does it matter
Essential thrombocythemia vs. early/prefibrotic myelofibrosis: Why does it matter
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DOI：
10.1016/j.beha.2014.07.004
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发表时间：
2014-06-01
影响因子：
2.1
通讯作者：
Barosi, Giovanni
中科院分区：
文献类型：
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作者：
Barosi, Giovanni
原发性血小板增多症 (ET) 和原发性骨髓纤维化 (PMF) 以及真性红细胞增多症 (PV) 是 Phildelphia 阴性 (Ph-neg) 经典骨髓增生性肿瘤 (MPN)。 ET 传统上通过血小板增多和缺乏相关骨髓 (BM) 纤维化来鉴定，而 PMF 则通过 BM 网状蛋白或胶原纤维化伴巨核细胞增生和发育不良以及髓外造血来鉴定。自 2001 年以来，这些诊断特征一直受到挑战，当时世界卫生组织 (WHO) 将一类新的患者纳入 PMF 领域，即早期/前纤维化 MF，其特征是 BM 中不存在相关的网状蛋白纤维化、双巨核细胞和粒细胞增殖以及巨核细胞发育不良。本综述的重点是总结早期/前纤维化骨髓纤维化的诊断不确定性、我们对该变异生物学理解的最新进展以及随之而来的转化意义。 (C) 2014 Elsevier Ltd. 保留所有权利。
Essential thrombocythemia (ET) and primary myelofibrosis (PMF), together with polycythemia vera (PV) are Phildelphia-negative (Ph-neg) classical myeloproliferative neoplasms (MPN). ET has been traditionally identified by thrombocytosis and absence of relevant bone marrow (BM) fibrosis, while PMF by BM reticulin or collagen fibrosis with megakaryocyte hyperplasia and dysplasia, and extramedullary hematopoiesis. These diagnostic profiles have been challenged since 2001 when the World Health Organization (WHO) has included in the domain of PMF a new category of patients, namely early/prefibrotic MF, characterized by the absence of relevant reticulin fibrosis in BM, dual megakaryocyte and granulocyte proliferation, and megakaryocyte dysplasia. This review is focused on summarizing the diagnostic uncertainties of early/prefibrotic myelofibrosis, recent advances in our understanding of the biology of the variant, and the accompanying translational implications. (C) 2014 Elsevier Ltd. All rights reserved.