Paediatric Department, Instituto Português de Oncologia de Lisboa Francisco Gentil EPE, Lisbon, Portugal.
Clinical Research Unit, Instituto Português de Oncologia de Lisboa Francisco Gentil EPE, Lisbon, Portugal.
Br J Haematol. 2016 Jul;174(2):280-91. doi: 10.1111/bjh.14048. Epub 2016 Mar 28.
Central Nervous System (CNS) thrombosis is a complication of acute lymphoblastic leukaemia (ALL) treatment that is potentially associated with significant morbidity and neurological sequelae. Its presumably multifactorial aetiology is poorly characterized. We conducted a single-centre, retrospective cohort study on 346 ALL paediatric patients (1-16 years old) treated with asparaginase intensive Dana Farber Cancer Institute (DFCI) protocols from 1998 to 2011. The incidence, risk factors and outcome of CNS thrombosis were evaluated. CNS thrombosis occurred in 3·8% (13/346) of the patients (95% confidence interval 2·0-6·3%). Twelve events were diagnosed during intensification, all of which resolved within 2 weeks without neurological sequelae or significant impact in survival. Obesity (body mass index above 95th percentile) and asparaginase formulation were the only factors associated with CNS thrombosis, with an increase in the odds of event in obese patients [odds ratio (OR) = 3·37; P = 0·064] and a reduction in patients receiving Erwinia asparaginase (OR = 0·12; P = 0·018). No association could be demonstrated for age, gender, DFCI risk-group, ALL phenotype, steroid or doxorubicin use, central venous line use or CNS radiotherapy. CNS thrombosis is a rare but manageable adverse event without significant sequelae or detrimental effects in survival. Increased awareness is recommended in obese patients particularly during intensive asparaginase use.
中枢神经系统(CNS)血栓形成是急性淋巴细胞白血病(ALL)治疗的一种并发症,可能与严重的发病率和神经后遗症有关。其多因素病因尚未得到很好的描述。我们对 1998 年至 2011 年间接受天冬酰胺酶强化 Dana-Farber 癌症研究所(DFCI)方案治疗的 346 例 ALL 儿科患者(1-16 岁)进行了一项单中心回顾性队列研究。评估了 CNS 血栓形成的发生率、危险因素和结果。346 例患者中有 3.8%(13/346)发生了 CNS 血栓形成(95%置信区间 2.0-6.3%)。强化治疗期间诊断出 12 例事件,所有事件均在 2 周内解决,无神经后遗症或对生存无重大影响。肥胖(体重指数高于第 95 百分位数)和天冬酰胺酶制剂是唯一与 CNS 血栓形成相关的因素,肥胖患者发生事件的几率增加[比值比(OR)=3.37;P=0.064],接受欧文氏菌天冬酰胺酶的患者减少[比值比(OR)=0.12;P=0.018]。年龄、性别、DFCI 风险组、ALL 表型、类固醇或阿霉素使用、中心静脉置管使用或 CNS 放疗与 CNS 血栓形成均无关联。CNS 血栓形成是一种罕见但可管理的不良事件,无明显后遗症或对生存无不利影响。建议肥胖患者,特别是在强化使用天冬酰胺酶期间,提高认识。