Abstract：
Myeloproliferative neoplasms (MPN) are a group of clonal haematological malignancies first described by Dameshek in 1957. The Philadelphia-negative MPN that will be described are polycythaemia vera (PV), essential thrombocythaemia (ET), pre-fibrotic myelofibrosis and primary myelofibrosis (PMF). The blood and bone marrow morphology are essential in diagnosis, for WHO classification, establishing a baseline, monitoring response to treatment and identifying changes that may indicate disease progression. The blood film changes may be in any of the cellular elements. The key bone marrow features are architecture and cellularity, relative complement of individual cell types, reticulin content and bony structure. Megakaryocytes are the most abnormal cell and key to classification, as their number, location, size and cytology are all disease-defining. Reticulin content and grade are integral to assignment of the diagnosis of myelofibrosis. Even with careful assessment of all these features, not all cases fit neatly into the diagnostic entities; there is frequent overlap reflecting the biological disease continuum rather than distinct entities. Notwithstanding this, an accurate morphologic diagnosis in MPN is crucial due to the significant differences in prognosis between different subtypes and the availability of different therapies in the era of novel agents. The distinction between \"reactive\" and MPN is also not always straightforward and caution needs to be exercised given the prevalence of \"triple negative\" MPN. Here we describe the morphology of MPN including comments on changes with disease evolution and with treatment.
摘要：
骨髓增殖性肿瘤（MPN）是Dameshek于1957年首次描述的一组克隆性血液恶性肿瘤。将描述的费城阴性MPN是真性多囊性贫血（PV），原发性血小板血症（ET），纤维化前骨髓纤维化和原发性骨髓纤维化（PMF）。血液和骨髓形态在诊断中至关重要，对于世界卫生组织的分类，建立基线，监测对治疗的反应，并确定可能表明疾病进展的变化。血膜变化可以在任何细胞元件中。关键的骨髓特征是结构和细胞结构，单个细胞类型的相对补体，网状蛋白含量和骨结构。巨核细胞是最异常的细胞，也是分类的关键，作为他们的号码，location,大小和细胞学检查都是疾病定义。网状蛋白含量和等级是骨髓纤维化诊断的组成部分。即使仔细评估了所有这些功能，并非所有病例都能完全适应诊断实体；经常有重叠反映生物学疾病的连续体，而不是不同的实体。尽管如此,MPN的准确形态学诊断至关重要,因为在新型药物时代,不同亚型之间的预后存在显著差异,而且不同治疗方法的可用性也存在显著差异.“反应性”和MPN之间的区别并不总是简单的，鉴于“三重阴性”MPN的患病率，需要谨慎行事。在这里，我们描述了MPN的形态，包括对疾病演变和治疗变化的评论。
