门冬酰胺酶相关儿童脑静脉窦血栓形成8例分析  

Analysis of 8 cases of asparaginase related cerebral venous sinus thrombosis in children

作　　者：魏会霞[1] 李白[1] 杨凤东[2] 苏淑芳[1] 魏林林[1] 丁艳杰 刘玉峰[1] 王叨[1] Wei Huixia;Li Bai;Yang Fengdong;Su Shufang;Wei Linlin;Ding Yanjie;Liu Yufeng;Wang Dao(Department of Hematology and Oncology,Children′s Hospital,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院儿童医院血液肿瘤科,郑州450052 [2]郑州大学第一附属医院神经外科,郑州450052

出　　处：《中华儿科杂志》2022年第6期567-572,共6页Chinese Journal of Pediatrics

基　　金：河南省医学科技攻关省部共建重点项目(SBGJ202002114)。

摘　　要：目的总结门冬酰胺酶(ASP)相关脑静脉窦血栓形成(CVST)患儿的临床特点、治疗及预后。方法回顾性分析2016年11月至2021年10月郑州大学第一附属医院收治的8例急性淋巴细胞白血病(ALL)或淋巴母细胞淋巴瘤(LBL)发生ASP相关CVST患儿的发病年龄、性别、首发症状、凝血功能、影像学特点、ASP的种类、治疗及转归等信息。结果8例CVST患儿均为男童,其中ALL 6例、LBL 2例,发病年龄5~15岁。6例发生在初次诱导缓解阶段。首发表现为癫痫大发作7例。磁共振成像联合磁共振静脉成像(MRV)显示均有上矢状窦血栓,合并脑出血5例。8例患儿D二聚体均增高。治疗上,3例患儿接受机械取栓+溶栓联合抗凝治疗,3例单纯抗凝治疗,2例未抗凝治疗。随访3个月MRV显示除2例未抗凝治疗患儿血栓部分吸收外,其他患儿血栓基本全部吸收,机械取栓+溶栓术联合抗凝治疗患儿血栓吸收最快。1例患儿死于ALL早期复发,7例存活患儿后续应用ASP无CVST反复或加重,除1例遗留左上肢肌力减低,余均无神经系统后遗症。结论ASP治疗相关CVST多发于大龄男性患儿,总体预后良好,好发于初次诱导缓解期,多以癫痫大发作为首发表现,上矢状窦为常见血栓部位,磁共振成像联合MRV可协助准确诊断,及时抗凝治疗安全有效,机械取栓+溶栓可快速再通血管。Objective To summarize the clinical features,treatment and prognosis of asparaginase(ASP)related cerebral venous sinus thrombosis(CVST).Methods Clinical profiles including age,sex,first symptoms,coagulation function,imaging findings,ASP type,treatment and prognosis of eight acute lymphoblastic leukemia(ALL)or lymphoblastic lymphoma(LBL)children with ASP related CVST at the Department of Pediatrics,First Affiliated Hospital of Zhengzhou University from November 2016 to October 2021 were analyzed retrospectively.Results Eight CVST children were all male,including 6 ALL and 2 LBL,with the onset age ranged from 5 to 15 years,6 cases occurred in the stage of first induction remission,and the initial symptom were mainly epileptic seizures(7 cases).Magnetic resonance imaging combined magnetic resonance venography(MRV)showed the most common site of venous sinus enlargement was superior sagittal sinus(8 cases).Secondary cerebral hemorrhage was found in 5 cases.D-dimer elevated on the day of onset in all cases.Three patients were treated with intravascular mechanical thrombectomy and thrombolysis combined with anticoagulant therapy,3 patients were treated with continuous anticoagulant therapy only,2 patients were not treated with anticoagulant therapy.MRV follow-up for 3 months showed that the thrombi in patients were almost completely absorbed except in 2 patients who were not treated with anticoagulant therapy.Thrombolysis combined with anticoagulant therapy was the fastest way for thrombosis absorption.Among 8 patients,1 died of early recurrence of ALL,and 7 patients accepted further asparaginase and no CVST recurrence or progression was found.There were no sequelae of nervous system except 1 patient with left upper limb muscle strength impairment.Conclusions ASP related CVST is more common in older male children and the prognosis is good.ASP related CVST occurred mostly in the stage of first induction remission,and most initial manifestation is epileptic seizure.The superior sagittal region is a common site of thrombus

关 键 词：天冬酰胺酶 儿童 静脉血栓形成 脑静脉 

分 类 号：R743[医药卫生—神经病学与精神病学]