Risk factors for early death in neonates with Down syndrome and transient leukaemia
Risk factors for early death in neonates with Down syndrome and transient leukaemia
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DOI：
10.1111/j.1365-2141.2008.07231.x
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发表时间：
2008-08-01
影响因子：
6.5
通讯作者：
Kojima, Seiji
中科院分区：
文献类型：
--
作者：
Muramatsu, Hideki;Kato, Koji;Kojima, Seiji
唐氏综合征（DS）新生儿的短暂性白血病（TL）的特征是外周血中短暂出现原始细胞并自发消退。一些TL患者由于器官衰竭而在早期死亡。回顾性研究了70例患有TL的DS患者，以确定与早期死亡（< 6个月）相关的临床和实验室特征。70例患者中有16例（22.9%）早期死亡。死亡的主要原因是器官衰竭，特别是肝脏和心肺衰竭。单因素分析显示，胎龄早（EGA）、高白色血细胞（WBC）计数（>= 100 × 10（9）/l）、外周血原始细胞百分比、天冬氨酸转氨酶（AST）升高、直接胆红素（DB）升高和低Apgar评分与生存率差显著相关。在多变量分析中，EGA、WBC计数和DB是不良结局的独立预测因素。设计了一个结合EGA和WBC计数的简单风险分层系统来预测不良结局。WBC计数低于100 x 10（9）/l的足月儿（EGA >= 37周）预后最好[7.7%（3/39）早死]，而WBC计数高于100 x 10（9）/l的早产儿（EGA < 37周）预后最差[54.5%（6/11）早死]。这种分层系统可能有助于识别需要早期治疗干预的高危患者。
Transient leukaemia (TL) in neonates with Down syndrome (DS) is characterized by the transient appearance of blast cells in the peripheral blood that resolves spontaneously. Some TL patients die at an early age due to organ failure. Seventy DS patients with TL were studied retrospectively to identify clinical and laboratory characteristics associated with early death (< 6 months of age). Sixteen of 70 patients (22.9%) died early. The main causes of death were organ failure, particularly hepatic and cardiopulmonary failure. On univariate analysis, early gestational age (EGA), high white blood cell (WBC) count (>= 100 x 10(9)/l), percentage of peripheral blasts, elevated aspartate transaminase (AST), elevated direct bilirubin (DB), and low Apgar score were significantly associated with poor survival. On multivariate analysis, EGA, WBC count, and DB were independent predictors of poor outcome. A simple risk stratification system combining EGA and WBC count was devised to predict poor outcome. Term infants (EGA >= 37 weeks) whose WBC count was lower than 100 x 10(9)/l had the best outcome [7.7% (3/39) died early], while preterm infants (EGA < 37 weeks) whose WBC count was higher than 100 x 10(9)/l had the worst outcome [54.5% (6/11) died early]. This stratification system may be useful for identifying high-risk patients who need early therapeutic interventions.