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目的:总结儿童急性早幼粒细胞白血病(APL)并发血栓的临床特点及预后。方法:回顾性分析2012年1月至2019年12月郑州大学第一附属医院收治的4例APL并发血栓患儿的临床资料,并以“急性早幼粒细胞白血病”“血栓”“acute promyelocytic leukemia” “thrombosis”为关键词,对中国知网、万方数据库、中国生物医学文献数据库、PubMed建库至2020年6月的相关文献进行检索,结合文献复习,对APL并发血栓的临床特点及预后进行总结。结果:4例患儿中男1例、女3例,年龄4~13岁。2例血栓发生于颅内血管,分别为颅内动脉及静脉窦血栓,表现为一侧肢体无力和抽搐;1例发生于脾脏动脉,导致脾脏梗死,表现为剧烈腹痛;1例发生于肝静脉,表现为腹痛、腹胀、腹腔积液。1例发生于诱导治疗前,3例发生于诱导治疗中。4例血栓经影像学检查确诊。经溶栓、抗凝或对症处理后,3例血管再通,症状缓解;合并肝静脉血栓的患儿死亡。检索文献未检索到中文儿童病例报道,11篇英文文献共报道11例儿童APL并发血栓病例。结合本组4例,15例中男9例,女6例,年龄3~16岁。15例患儿中,8例发生于动脉,7例发生于静脉。6例发生于颅内,3例发生于下肢,3例发生于肝脏,2例发生于脾脏,1例发生于心脏冠状动脉。10例发生于诱导治疗前,5例发生于诱导治疗中。15例患儿经手术、溶栓、抗凝或对症处理后,10例血管再通,症状缓解;5例死亡,发生肝静脉血栓的3例患儿均发生死亡。结论:儿童APL合并血栓可发生于诱导治疗前、后。动、静脉均可累及。发生部位广泛,临床表现无特异性。影像学检查可确定诊断。治疗后大多血管再通,预后良好,合并肝静脉血栓者病死率高。“,”Objective:To investigate the clinical characteristics and prognosis of children with acute promyelocytic leukemia (APL) complicated with thrombosis.Methods:The clinical profiles of four APL patients complicated with thrombosis treated at the First Affiliated Hospital of Zhengzhou University between January 2012 and December 2019 were reviewed. Literature search and review covered the China national knowledge infrastructure, Wanfang database, China biology medicine disc and PubMed using the key words of “acute promyelocytic leukemia” and “thrombosis” up to June 2020.Results:Four patients included one male and three females with an age range of 4-13 years. In two patients, thrombosis occurred intracranially, both patients presented with seizure and hemiplegia. In one patient, thrombosis occurred in spleen, the patient presented with severe abdominal pain. In the last patient, thrombosis occurred in liver, the patient presented with abdominal pain, distension and ascites. Thrombosis occurred during the course of retinoic acid treatment in three patients, it occurred before the initiation of the treatment in one patient. Thrombosis was confirmed by imaging examination in all four patients. After thrombolytic and anticoagulation treatment, the vessels were recanalized and the symptoms were alleviated in 3 cases, the fourth patient died of hepatic venous thrombosis. Literature search identified no similar reports in Chinese journals, 11 case reports were found in English journals. In these 15 patients, 9 were male and 6 females with an age range of 3-16 years. Thrombus located in brain in 6 cases, in lower limbs for 3 cases, in liver for 3 cases, in spleen in 2 cases and in coronary artery for 1 case. In 10 cases thrombosis occurred before the induce treatment and in 5 cases occurred during the induction treatment. After surgery, thrombolytic and anticoagulation treatment vascular recanalization was observed in 10 patients, and in those patients, the symptoms were relieved, death occurred in 5 patients, including three patients with hepatic venous thrombosis.Conclusions:Thrombosis may occur before or during the treatment for APL. Both arterial and venous thrombosis may occur in patients with APL. Imaging examination is a reliable method for its diagnosis. After treatment, most patients had good prognosis with recanalization of blood vessels, but the mortality rate was high for patients with hepatic venous thrombosis.