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Abstract：
Juvenile myelomonocytic leukemia (JMML) is a disorder characterized by the simultaneous presence of myeloproliferative and myelodysplastic features, primarily affecting infants and young children. Due to the heterogeneous genetic background among patients, the current clinical and laboratory prognostic features are insufficient for accurately predicting outcomes. Thus, there is a pressing need to identify novel prognostic indicators. Red cell distribution width (RDW) is a critical parameter reflecting the variability in erythrocyte size. Recent studies have emphasized that elevated RDW serves as a valuable predictive marker for unfavorable outcomes across various diseases. However, the prognostic role of RDW in JMML remains unclear. Patients with JMML from our single-center cohort between January 2008 and December 2019 were included. Overall, 77 patients were eligible. Multivariate Cox proportional hazard models showed that patients with red cell distribution width coefficient of variation (RDW-CV) >17.35% at diagnosis were susceptible to much worse overall survival rate (hazard ratio [HR] = 5.22, confidence interval [CI] = 1.50-18.21, P = .010). Besides, the combination of RDW elevation and protein phosphatase non-receptor type 11 (PTPN11) mutation was likely to predict a subgroup with the worst outcomes in our cohort. RDW is an independent prognostic variable in JMML subjects. RDW may be regarded as an inexpensive biomarker to predict the clinical outcome in patients with JMML.
摘要：
幼年型粒单核细胞白血病（JMML）是一种以同时存在骨髓增生和骨髓增生异常为特征的疾病。主要影响婴儿和幼儿。由于患者遗传背景的异质性,目前的临床和实验室预后特征不足以准确预测结局.因此，迫切需要确定新的预后指标.红细胞分布宽度（RDW）是反映红细胞大小变异性的关键参数。最近的研究强调，RDW升高是各种疾病不利结果的有价值的预测标志物。然而,RDW在JMML中的预后作用尚不清楚.纳入2008年1月至2019年12月来自我们单中心队列的JMML患者。总的来说,77名患者符合条件。多因素Cox比例风险模型显示，诊断时红细胞分布宽度变异系数（RDW-CV）>17.35%的患者总生存率更差（风险比[HR]=5.22，置信区间[CI]=1.50-18.21，P=.010）。此外,RDW升高和蛋白磷酸酶非受体11型(PTPN11)突变联合可能预测我们队列中结局最差的亚组.RDW是JMML受试者的独立预后变量。RDW可以被认为是预测JMML患者临床结果的廉价生物标志物。
