文章：

骨髓纤维化在真性红细胞增多症中的预后影响：IWG-MRT研究的验证及其他观察

Prognostic impact of bone marrow fibrosis in polycythemia vera: validation of the IWG-MRT study and additional observations

原文发布日期：2017-03-10

DOI: 10.1038/bcj.2017.17

类型: Original Article

开放获取: 是

英文摘要：

In 2012, the International Working Group for Myeloproliferative Neoplasms (MPN) Research and Treatment (IWG-MRT) reported an associations between mild bone marrow (BM) fibrosis (⩾grade 1) in polycythemia vera (PV) and a lower incidence of thrombosis during the clinical course and a higher risk of fibrotic progression. The objective in the current study of 262 patients with PV was to validate these observations and also identify other risk factors for myelofibrosis-free survival (MFFS). About 127 (48%) patients displayed ⩾grade 1 reticulin fibrosis at the time of diagnosis; presenting clinical and laboratory features were not significantly different between patients with or without BM fibrosis. In univariate analysis, BM fibrosis had no significant impact on overall, leukemia-free or thrombosis-free survival, whereas a significant association was noted for MFFS (P=0.009, hazard ratio 2.9; 95% confidence interval 1.32–6.78); other risk factors for MFFS included leukocytosis ⩾15 × 109/l, presence of palpable splenomegaly and abnormal karyotype. During multivariable analysis, leukocytosis ⩾15 × 109/l, palpable splenomegaly and ⩾grade 1 BM reticulin fibrosis remained significant. The current study validates the previously observed association between ⩾grade 1 BM reticulin fibrosis in PV and subsequent fibrotic progression, and identifies leukocytosis and palpable splenomegaly as additional risk factors for fibrotic progression; additional studies are required to clarify the impact of BM fibrosis on thrombosis and that of abnormal karyotype on MFFS.

摘要翻译： 

2012年，骨髓增殖性肿瘤研究与治疗国际工作组（IWG-MRT）报告显示，真性红细胞增多症（PV）患者出现轻度骨髓纤维化（≥1级）与临床过程中较低的血栓发生率及较高的纤维化进展风险存在关联。本研究旨在通过对262例PV患者的分析验证上述发现，并识别无骨髓纤维化生存（MFFS）的其他风险因素。诊断时127例（48%）患者存在≥1级网状蛋白纤维化；伴或不伴骨髓纤维化患者的临床及实验室特征无显著差异。单因素分析显示，骨髓纤维化对总生存期、无白血病生存期或无血栓生存期无显著影响，但与MFFS存在显著关联（P=0.009，风险比2.9；95%置信区间1.32-6.78）；MFFS的其他风险因素包括白细胞计数≥15×10⁹/L、可触及脾肿大及核型异常。多因素分析确认白细胞计数≥15×10⁹/L、可触及脾肿大和≥1级骨髓网状蛋白纤维化仍具显著意义。本研究验证了既往观察到的PV患者≥1级骨髓网状蛋白纤维化与后续纤维化进展的关联，并明确白细胞增多和可触及脾肿大是纤维化进展的附加风险因素；需进一步研究阐明骨髓纤维化对血栓形成的影响及异常核型对MFFS的作用。

原文链接：

Prognostic impact of bone marrow fibrosis in polycythemia vera: validation of the IWG-MRT study and additional observations