培门冬酶治疗急性淋巴细胞白血病致颅内静脉窦血栓形成3例并文献复习  

Cerebral venous sinus thrombosis in acute lymphoblastic leukemia treated by pegaspargase:report of 3 cases and review of literature

作　　者：姚振 钱崇升 曹涵钰 张彤彤[1] 宫文洁 周海侠[1] 徐明珠[1] 薛胜利[1] Yao Zhen;Qian Chongsheng;Cao Hanyu;Zhang Tongtong;Gong Wenjie;Zhou Haixia;Xu Mingzhu;Xue Shengli(Department of Hematology,the First Affiliated Hospital of Soochow University,Suzhou 215006,China)

机构地区：[1]苏州大学附属第一医院血液内科,苏州215006

出　　处：《白血病．淋巴瘤》2023年第12期723-728,共6页Journal of Leukemia & Lymphoma

摘　　要：目的探讨培门冬酶治疗急性淋巴细胞白血病(ALL)致颅内静脉窦血栓形成(CVST)的诊断、治疗、临床特征及潜在高危因素。方法回顾性分析2021年3月、11月和2022年9月于苏州大学附属第一医院接受培门冬酶治疗出现CVST的3例ALL患者病史、诊疗经过、实验室检查和影像学检查结果,并复习相关文献。结果3例患者均为女性,年龄15~35岁,其中2例为B-ALL患者,1例为T-ALL患者。均于培门冬酶化疗后出现神经系统症状,后经影像学检查确诊为CVST。培门冬酶治疗期间,2例口服炔诺酮,1例行引产术及清宫术,3例患者的性激素水平均有非生理性改变。CVST主要病变部位集中在上矢状窦、横窦及乙状窦,其中1例合并脑出血。发生血栓时,患者的纤维蛋白原(Fib)、抗凝血酶原Ⅲ(ATⅢ)活性、蛋白C活性和蛋白S活性较前明显下降,D-二聚体明显升高,狼疮抗凝物及抗心磷脂抗体均为阴性。血栓治疗均以抗凝为主,1例行溶栓术。2例无神经系统后遗症,1例有肌力减退后遗症。结论ALL患者使用培门冬酶化疗期间出现神经系统症状时应警惕CVST,CVST诊断主要依靠头颅影像学检查。血栓治疗以抗凝为主,部分患者可行溶栓及介入取栓治疗,很少有神经系统后遗症。第二代孕酮类药物的使用及性激素的非生理性波动可能是CVST潜在的危险因素。Objective To investigate the diagnosis,treatment,clinical characteristics and potential high-risk factors of cerebral venous sinus thrombosis(CVST)during the treatment of acute lymphoblastic leukemia(ALL)with pegaspargase.Methods The medical history,diagnosis and treatment process,laboratory examination and imaging examination results of 3 ALL patients with CVST during pegaspargase treatment in the First Affiliated Hospital of Suzhou University in March and November 2021 and September 2022 were retrospectively analyzed,and the relevant literature was reviewed.Results Three patients were all female,with the aged between 15 and 35 years old,including 2 cases of B-ALL and 1 case of T-ALL.All patients developed nervous system symptoms after pegaspargase chemotherapy,and were diagnosed as CVST by imaging examination.During the pegaspargase treatment,2 patients took norethisterone,and 1 patient underwent induced labor and curettage.The levels of sexual hormones in the 3 patients had non-physiological changes.The main CVST lesions were located in the superior sagittal sinus,transverse sinus and sigmoid sinus.One patient had cerebral hemorrhage at the same time.When thrombus occurred,the fibrinogen(Fib),antithrombinⅢ(ATⅢ)activity,protein C activity and protein S activity of the patients were significantly lower than those before,D-dimer was significantly higher,and lupus anticoagulant and anticardiolipin antibody were negative.The thrombosis treatment was mainly anticoagulation,and 1 patient underwent thrombolysis.Two patients had no sequelae of nervous system,and 1 patient had the sequelae of muscle weakness.Conclusions Patients with ALL should be alert to the occurrence of CVST when they have nervous system symptoms during pegaspargase chemotherapy.The diagnosis of CVST mainly depends on cranial imaging.Anticoagulation is the main thrombosis treatment,thrombolysis and interventional thrombectomy are feasible for some patients,with few neurological sequelae.The use of second-generation progesterone drugs and the non

关 键 词：前体细胞淋巴母细胞白血病淋巴瘤 培门冬酶 窦血栓形成 颅内 孕酮同源物 危险因素 

分 类 号：R733.71[医药卫生—肿瘤]