Vener Claudia, Fracchiolla Nicola Stefano, Gianelli Umberto, Calori Rossella, Radaelli Franca, Iurlo Alessandra, Caberlon Sabrina, Gerli Giancarla, Boiocchi Leonardo, Deliliers Giorgio Lambertenghi
Ematologia I, Centro Trapianti di Midollo, University of Milan, Milano, Italy.
Blood. 2008 Feb 15;111(4):1862-5. doi: 10.1182/blood-2007-09-112953. Epub 2007 Nov 20.
Various clinical prognostic scoring systems (PSSs) have been suggested as means of selecting high-risk chronic idiopathic myelofibrosis (CIMF) patients at diagnosis. The WHO has recently proposed strict diagnostic criteria for CIMF, and the European consensus for bone marrow fibrosis (BMF) grading recommends 4 classes. It has been suggested that BMF grading may play a prognostic role in CIMF, but it has never been compared with the other PSSs in the same patients. We tested a prognostic model for overall survival (OS) based on the WHO criteria and BMF grading in 113 consecutive patients with chronic myeloproliferative disorders (98 with CIMF and 15 with postpolycythemic myelofibrosis), and compared the findings with those of PSSs. The results showed that our model is significantly associated with different OSs and, unlike the other PSSs, clearly discriminates the OS of intermediate- and high-risk patients.
各种临床预后评分系统（PSS）已被提出作为在诊断时选择高危慢性特发性骨髓纤维化（CIMF）患者的手段。世界卫生组织（WHO）最近提出了CIMF的严格诊断标准，欧洲骨髓纤维化（BMF）分级共识推荐分为4类。有人提出BMF分级可能在CIMF中起预后作用，但从未在同一患者中与其他PSS进行比较。我们基于WHO标准和BMF分级对113例连续的慢性骨髓增殖性疾病患者（98例CIMF患者和15例真性红细胞增多症后骨髓纤维化患者）的总生存期（OS）进行了预后模型测试，并将结果与PSS的结果进行了比较。结果表明，我们的模型与不同的OS显著相关，并且与其他PSS不同，能够清晰地区分中高危患者的OS。
