文章：

2016年世界卫生组织骨髓增殖性肿瘤分类与诊断标准：文件摘要与深入讨论

The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion

原文发布日期：2018-02-09

DOI: 10.1038/s41408-018-0054-y

类型: Review Article

开放获取: 是

英文摘要：

The new edition of the 2016 World Health Organization (WHO) classification system for tumors of the hematopoietic and lymphoid tissues was published in September 2017. Under the category of myeloproliferative neoplasms (MPNs), the revised document includes seven subcategories: chronic myeloid leukemia, chronic neutrophilic leukemia, polycythemia vera (PV), primary myelofibrosis (PMF), essential thrombocythemia (ET), chronic eosinophilic leukemia-not otherwise specified and MPN, unclassifiable (MPN-U); of note, mastocytosis is no longer classified under the MPN category. In the current review, we focus on the diagnostic criteria for JAK2/CALR/MPL mutation-related MPNs: PV, ET, and PMF. In this regard, the 2016 changes were aimed at facilitating the distinction between masked PV and JAK2-mutated ET and between prefibrotic/early and overtly fibrotic PMF. In the current communication, we (i) provide practically useful resource tables and graphs on the new diagnostic criteria including outcome, (ii) elaborate on the rationale for the 2016 changes, (iii) discuss the complementary role of mutation screening, (iv) address ongoing controversies and propose solutions, (v) attend to the challenges of applying WHO criteria in routine clinical practice, and (vi) outline future directions from the perspectives of the clinical pathologist.

摘要翻译： 

2016年版世界卫生组织（WHO）造血与淋巴组织肿瘤分类系统于2017年9月发布。在骨髓增殖性肿瘤（MPN）类别中，修订后的文件包含七个亚类：慢性髓系白血病、慢性中性粒细胞白血病、真性红细胞增多症（PV）、原发性骨髓纤维化（PMF）、原发性血小板增多症（ET）、非特指型慢性嗜酸性粒细胞白血病以及未分类MPN（MPN-U）；值得注意的是，肥大细胞增多症不再归类于MPN范畴。本文重点探讨JAK2/CALR/MPL突变相关MPN（PV、ET和PMF）的诊断标准。2016版修订旨在更好区分隐匿性PV与JAK2突变ET，以及纤维化前/早期阶段与明显纤维化期PMF。本文将：（i）提供涵盖预后的新诊断标准实用资源表和图示；（ii）阐述2016版修订的理论依据；（iii）讨论突变筛查的互补作用；（iv）厘清现存争议并提出解决方案；（v）关注WHO标准在临床实践中的应用挑战；（vi）从临床病理学家视角展望未来研究方向。

原文链接：

The 2016 WHO classification and diagnostic criteria for myeloproliferative neoplasms: document summary and in-depth discussion