摘要目的:探讨儿童急性淋巴细胞白血病（ALL）合并脑静脉血栓形成（CVT）的临床特点及诊疗对策。方法:纳入2011年1月至2019年10月在中山大学孙逸仙纪念医院儿科住院并接受化疗的ALL合并CVT的14例患儿的临床资料进行回顾性分析，分析其临床表现、凝血功能、影像学特点、治疗方案。结果:CVT发生率为2.8%（14/505）。14例患儿男11例、女3例，CVT发病年龄10（3~14）岁。11例发生在诱导缓解阶段，急性期表现为抽搐（9例）、意识障碍（6例）及头痛（4例）。凝血功能检测发现，8例发生CVT前抗凝血酶Ⅲ活性低于60%，8例出现症状当天D二聚体升高。动静脉造影示单部位（9例）或多部位（5例）静脉窦内充盈缺损，静脉窦增宽，最常见部位为上矢状窦（10例），5例继发颅内出血。抗凝治疗包括联合及序贯使用低分子肝素（LMWH）和华法林，分别为9例和2例，单用LMWH3例，再次使用门冬酰胺酶无CVT反复或加重。结论:ALL诱导缓解治疗中继发性凝血功能紊乱是合并CVT主要因素，需积极监测、早期预防，动静脉造影可准确诊断，LMWH和华法林抗凝治疗预后良好。

abstractsObjective:To explore the clinical characteristics and management of childhood acute lymphoblastic leukemia (ALL) complicated with cerebral venous thrombosis (CVT).Methods:The clinical data of 14 ALL children complicated with CVT who were admitted to Department of Pediatrics of Sun Yat-sen Memorial Hospital and underwent chemotherapy from January 2011 to October 2019 were collected retrospectively. The clinical manifestations, coagulation function, imaging findings, treatment plan and prognosis of patients were analyzed.Results:CVT was diagnosed in 14 (2.8%, 14/505) cases, with a median age of 10 (3-14) years at onset, 11 cases occurred in the stage of induction remission, and the acute onsets were mainly characterized by convulsions (9 cases), consciousness disorders (6 cases) and headache (4 cases). Coagulation function test showed that, before the CVT, antithrombin Ⅲ activity was lower than 60% in 8 cases, D-dimer elevated on the day of onset in 8 cases. Arteriovenous angiography showed filling defects in single (9 cases) or multiple (5 cases) venous sinuses. The most common site of venous sinus enlargement was superior sagittal sinus (10 cases). Secondary cerebral hemorrhage was found in 5 cases. Anticoagulation therapy included combination of low-molecular-weight heparin (LMWH) and warfarin in 9 cases, sequential application of LMWH and warfarin in 2 cases, and LMWH alone in 3 cases. Patients accepted further asparaginase and no CVT recurrence or progression was found.Conclusions:The secondary coagulation dysfunction during induction remission chemotherapy is the major risk factor for CVT in ALL, which needs active monitoring and early prevention. Arteriovenous angiography can diagnose accurately, and the prognosis of anticoagulant therapy with LMWH and warfarin is optimistic.