Prognostic impact of bone marrow fibrosis in polycythemia vera: validation of the IWG-MRT study and additional observations.
Prognostic impact of bone marrow fibrosis in polycythemia vera: validation of the IWG-MRT study and additional observations.
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DOI：
10.1038/bcj.2017.17
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发表时间：
2017-03-10
影响因子：
12.8
通讯作者：
Tefferi A
中科院分区：
文献类型：
--
作者：
Barraco D;Cerquozzi S;Hanson CA;Ketterling RP;Pardanani A;Gangat N;Tefferi A
2012年，骨髓增生性肿瘤（MPN）研究和治疗国际工作组（IWG-MRT）报告了真性红细胞增多症（PV）中轻度骨髓（BM）纤维化（大于或小于1级）与临床过程中较低的血栓发生率和较高的纤维化进展风险之间的关联。目前对262例PV患者的研究目的是验证这些观察结果，并确定无骨髓纤维化生存（MFFS）的其他危险因素。约127（48%）患者在诊断时显示出小于或等于1级网状蛋白纤维化；BM纤维化患者的临床和实验室表现无显著差异。在单因素分析中，BM纤维化对总体、无白血病或无血栓形成生存无显著影响，而MFFS有显著关联（P=0.009，风险比2.9；95%可信区间1.32-6.78）；MFFS的其他危险因素包括白细胞增生大于或等于15 × 109/l、存在可触及的脾肿大和核型异常。在多变量分析期间，白细胞增生大于或等于15 × 109/l、可触及脾肿大和小于或等于1级BM网状蛋白纤维化仍然显著。目前的研究验证了先前观察到的PV中小于1级BM网状蛋白纤维化和随后的纤维化进展之间的关联，并确定白细胞增多和可触摸脾肿大是纤维化进展的额外危险因素；需要进一步的研究来阐明BM纤维化对血栓形成的影响以及异常核型对MFFS的影响。
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.