Research Foundation, Papa Giovanni XXIII Hospital, Bergamo, Italy.
Institute of pathology, University of Cologne, Cologne, Germany.
Am J Hematol. 2016 Jun;91(4):430-3. doi: 10.1002/ajh.24288.
In myeloproliferative neoplasms (MPNs), controversy persists regarding the usefulness and reproducibility of bone marrow (BM) features. Disagreements concerning the WHO classification are mainly focused on the discrimination between essential thrombocythemia (ET) and prefibrotic/early primary myelofibrosis (prePMF) and prodromal polycythemia vera (PV). Criticism mostly refers to lack of standardization of distinctive BM features precluding correct morphological pattern recognition. The distinction between WHO-defined ET and prePMF is not trivial because outcome is significantly worse in prePMF. Morphology was generally considered to be non-specific for the diagnosis of PV. Recent studies have revealed under-diagnosis of morphologically and biologically consistent PV.
在骨髓增殖性肿瘤（MPNs）中，关于骨髓（BM）特征的有用性和可重复性仍然存在争议。关于世界卫生组织（WHO）分类的分歧主要集中在原发性血小板增多症（ET）和纤维化前期/早期原发性骨髓纤维化（prePMF）以及前驱性红细胞增多症（PV）的鉴别上。批评主要是针对缺乏独特的 BM 特征的标准化，从而妨碍了正确的形态学模式识别。WHO 定义的 ET 和 prePMF 之间的区别并非微不足道，因为 prePMF 的预后明显更差。形态学通常被认为对 PV 的诊断不具有特异性。最近的研究表明，形态学和生物学上一致的 PV 存在漏诊。
