Duong Vu H, Padron Eric, Al Ali Najla H, Lancet Jeffrey E, Hall Jeff, Kwok Brian, Zhang Ling, Epling-Burnette Pearlie K, List Alan F, Komrokji Rami S
University of Maryland School of Medicine and Greenebaum Comprehensive Cancer Center, 22 S. Greene Street, S9D04B, Baltimore, MD, 21201, USA.
Department of Malignant Hematology, H Lee Moffitt Cancer Center & Research Institute, Tampa, FL, USA.
Ann Hematol. 2018 Feb;97(2):247-254. doi: 10.1007/s00277-017-3186-4. Epub 2017 Nov 22.
The prognostic value of peripheral blasts (PB) is not well-studied in patients with myelodysplastic syndromes (MDS). We evaluated the impact of PB on overall survival (OS) and transformation to acute myeloid leukemia (AML) in a large cohort. The MDS database at the Moffitt Cancer Center was retrospectively reviewed to identify patients with ≥ 1% PB (PB-MDS) and those without PB (BM-MDS). We also assessed the correlation between PB and gene mutations. One thousand seven hundred fifty-eight patients were identified, among whom 13% had PB near the time of diagnosis. PB-MDS patients were more likely to be younger with trilineage cytopenia, complex karyotype, higher-risk disease, transfusion dependence, and therapy-related MDS. The rate of AML transformation was 49 vs. 26% (p < 0.005) and median OS was 16.5 vs. 45.8 months (p < 0.005) in the PB-MDS and BM-MDS groups, respectively. In Cox regression analysis, the presence of PB was an independent prognostic covariate for OS, HR 1.57 (95% CI 1.2-2). Among 51 patients with an available gene panel, the rate of ≥ 1 gene mutation in the PB-MDS group (n = 4) was 100% compared to 81% in the BM-MDS group (n = 47). The presence of PB in MDS is an adverse independent prognostic variable that refines prognostic discrimination.
外周血原始细胞（PB）在骨髓增生异常综合征（MDS）患者中的预后价值尚未得到充分研究。我们在一个大型队列中评估了PB对总生存期（OS）和转化为急性髓系白血病（AML）的影响。对莫菲特癌症中心的MDS数据库进行回顾性分析，以确定外周血原始细胞≥1%的患者（PB-MDS）和外周血无原始细胞的患者（BM-MDS）。我们还评估了PB与基因突变之间的相关性。共纳入1758例患者，其中13%在诊断时外周血存在原始细胞。PB-MDS患者更年轻，更易出现三系血细胞减少、复杂核型、高危疾病、输血依赖和治疗相关MDS。PB-MDS组和BM-MDS组的AML转化率分别为49%和26%（p<0.005），中位OS分别为16.5个月和45.8个月（p<0.005）。在Cox回归分析中，PB的存在是OS的独立预后协变量，风险比（HR）为1.57（95%置信区间[CI]为1.2 - 2）。在51例有可用基因检测结果的患者中，PB-MDS组（n = 4）中≥1个基因突变的发生率为100%，而BM-MDS组（n = 47）为81%。MDS患者外周血存在原始细胞是一个不良的独立预后变量，可改善预后判别。
