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以布加综合征为初始表现的儿童急性早幼粒细胞白血病1例及文献复习

Study on a case of childhood acute promyelocytic leukemia with Budd-Chiari syndrome as the initial manifestation and literature review
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摘要 报告临床上较为罕见的1例以布加综合征(BCS)为初始表现的儿童急性早幼粒细胞白血病(APL)患者。患儿,女,6岁3个月,以呕吐、头痛及腹痛起病,其后出现全血细胞减少。完善腹部大血管的彩超提示下腔静脉肝后段、肝右静脉及肝左静脉稍强回声团,考虑血栓,其中肝右静脉闭塞可能,门静脉壁不光整。腹部计算机断层扫描(CT)提示肝脏外周密度稍偏低密度欠均匀,肝段下腔静脉及肝静脉显示欠佳。根据骨髓穿刺报告结果,患儿最总确诊为急性早幼粒细胞白血病。本研究对布加综合征为初始表现的儿童急性早幼粒细胞白血病的临床特点、诊断及治疗等进行分析,以提高儿科临床医生对该病的认识,及时作出诊断和合理的干预,以提高治疗的成功率。 This paper is intended to report a rare clinical case of childhood acute promyelocytic leukemia(APL)with Budd-Chiari syndrome(BCS)as the initial manifestation.The child,a female who aged 6 years and 3 months,initially manifested by vomiting,headache and abdominal pain,followed by pancytopenia.Color Doppler ultrasound of the large abdominal vessels suggested a slightly strong echogenic mass in the posterior segment of the inferior vena cava,the right hepatic vein and the left hepatic vein,which was considered thrombosis with possible occlusion of the right hepatic vein,and it was found that the portal vein wall was not smooth.Computerized tomographic scanning(CT)of the abdomen suggested that the peripheral density of the liver was slightly lower with uneven distribution,and that the inferior vena cava and hepatic vein of the hepatic segment were poorly displayed.According to the results of the bone marrow aspiration report sheet,the child was finally diagnosed with APL.This study analyzes the clinical characteristics,diagnosis and treatment of childhood APL with BCS as the initial manifestation,so as to improve the understanding of the pediatric clinicians about this disease and make timely diagnosis and reasonable interventions for higher success rate of treatment.
作者 徐灵均 陈天兵 陈剑 刘洪 XU Lingjun;CHEN Tianbing;CHEN Jian;LIU Hong(Department of Emergency,Jiangxi Provincial Children′s Hospital,Nanchang330006,China;Department of Functional Section,Jiangxi Provincial Children′s Hospital,Nanchang330006,China;Department of Blood Specialty,Jiangxi Provincial Children′s Hospital,Nanchang330006,China)
出处 《中国现代医生》 2022年第1期177-180,共4页 China Modern Doctor
基金 江西省卫生健康委科技计划项目(202130933)。
关键词 布加综合征 儿童 急性早幼粒细胞白血病 介入治疗 Budd-Chiari syndrome Child Acute promyelocytic leukemia Interventional treatment
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