文章：

诊断时淋巴细胞减少在骨髓增生异常综合征中非常普遍，并且在IPSS-R低危患者中具有独立的负面预后价值

Lymphopenia at diagnosis is highly prevalent in myelodysplastic syndromes and has an independent negative prognostic value in IPSS-R-low-risk patients

原文发布日期：2019-08-09

DOI: 10.1038/s41408-019-0223-7

类型: Article

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英文摘要：

Lymphopenia is associated with an increased mortality in several medical conditions. Its prognostic impact in myelodysplastic syndromes (MDS) is less well studied. Hence, we analyzed 1023 patients from the Düsseldorf MDS-registry with regard to the absolute lymphocyte count (ALC) at diagnosis. An ALC below the median of the population (1.2 × 109/l) was associated with lower counts of neutrophils (median 1.35 vs. 1.92 × 109/l, p < 0.001) and platelets (median 100 vs. 138 × 109/l, p < 0.001) and with a significant lower overall survival in univariate analysis (whole cohort: median 36 vs. 46 months, p = 0.016; 721 patients without hematopoietic stem cell transplantation or induction chemotherapy: median 36 vs. 56 months, p = 0.001). For low-risk MDS according to IPSS-R, an ALC < 1.2 × 109/l was of additional prognostic value in a multivariate Cox regression model together with age (< or ≥65 years) and LDH (< or ≥normal value of 240 U/l; HR 1.46, 95% CI: 1.03–2.08, p = 0.033). These data support the hypothesis of subtle but clinical relevant changes of the adaptive immune system in MDS. Further studies are necessary to identify the ALC cut-off best suitable for prognostication and the mechanisms responsible for the impairment of lymphoid homeostasis in MDS.
 

摘要翻译： 

淋巴细胞减少症与多种疾病死亡率升高相关，但其在骨髓增生异常综合征（MDS）中的预后意义研究较少。为此，我们分析了杜塞尔多夫MDS登记库中1023例患者的初诊绝对淋巴细胞计数（ALC）。当ALC低于人群平均值（1.2×10⁹/L）时，患者中性粒细胞（中位值1.35 vs. 1.92×10⁹/L, p<0.001）与血小板计数（中位值100 vs. 138×10⁹/L, p<0.001）均较低，且单因素分析显示总生存期显著缩短（整体队列：中位生存期36 vs. 46个月, p=0.016；排除造血干细胞移植或诱导化疗的721例患者：中位生存期36 vs. 56个月, p=0.001）。在IPSS-R评分系统的低危MDS患者中，联合年龄（＜或≥65岁）和乳酸脱氢酶（＜或≥正常值240 U/L）的多因素Cox回归模型显示，ALC＜1.2×10⁹/L具有独立预后价值（风险比1.46，95%置信区间：1.03-2.08, p=0.033）。这些数据印证了适应性免疫系统在MDS中存在轻微但具临床意义改变的假说。未来需进一步研究确定最佳预后ALC临界值，并阐明MDS中淋巴稳态失衡的作用机制。

原文链接：

Lymphopenia at diagnosis is highly prevalent in myelodysplastic syndromes and has an independent negative prognostic value in IPSS-R-low-risk patients