{"title":"EVI1阳性儿童慢性粒细胞白血病加速期1例报告及文献复习","authors":"C. Zhuang, Xiuxia Chen, Pingping Wei, Liang Hui","doi":"10.3760/CMA.J.ISSN.1006-9801.2019.12.013","DOIUrl":null,"url":null,"abstract":"目的 \n提高对EVI1基因阳性的儿童慢性粒细胞白血病加速期(CML-AP)的认识。 \n \n \n方法 \n回顾性分析青岛市妇女儿童医院2019年1月收治的1例EVI1基因阳性的儿童CML-AP患者的临床资料,并复习相关文献。 \n \n \n结果 \n患儿,男性,14岁,确诊后,加用国产伊马替尼联合小剂量化疗方案,达血液学完全缓解。BCR-ABL p210定量降至4.66%,EVI1定量降至正常(0.01%)。 \n \n \n结论 \n儿童CML-AP罕见,临床特征主要为白细胞计数明显升高和巨脾。国产伊马替尼联合小剂量化疗方案对EVI1基因阳性的儿童CML-AP治疗有效,远期疗效尚需密切随访。","PeriodicalId":9505,"journal":{"name":"肿瘤研究与临床","volume":null,"pages":null},"PeriodicalIF":0.0000,"publicationDate":"2019-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Childhood chronic myeloid leukemia in accelerated phase with EVI1 positive: report of one case and review of literature\",\"authors\":\"C. Zhuang, Xiuxia Chen, Pingping Wei, Liang Hui\",\"doi\":\"10.3760/CMA.J.ISSN.1006-9801.2019.12.013\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"目的 \\n提高对EVI1基因阳性的儿童慢性粒细胞白血病加速期(CML-AP)的认识。 \\n \\n \\n方法 \\n回顾性分析青岛市妇女儿童医院2019年1月收治的1例EVI1基因阳性的儿童CML-AP患者的临床资料,并复习相关文献。 \\n \\n \\n结果 \\n患儿,男性,14岁,确诊后,加用国产伊马替尼联合小剂量化疗方案,达血液学完全缓解。BCR-ABL p210定量降至4.66%,EVI1定量降至正常(0.01%)。 \\n \\n \\n结论 \\n儿童CML-AP罕见,临床特征主要为白细胞计数明显升高和巨脾。国产伊马替尼联合小剂量化疗方案对EVI1基因阳性的儿童CML-AP治疗有效,远期疗效尚需密切随访。\",\"PeriodicalId\":9505,\"journal\":{\"name\":\"肿瘤研究与临床\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2019-12-28\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"肿瘤研究与临床\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.3760/CMA.J.ISSN.1006-9801.2019.12.013\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"Medicine\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"肿瘤研究与临床","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.3760/CMA.J.ISSN.1006-9801.2019.12.013","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"Medicine","Score":null,"Total":0}
引用次数: 0
摘要
Objective: To improve understanding of the accelerated phase of chronic myeloid leukemia (CML-AP) in children with EVI1 gene positivity. Method: A retrospective analysis was conducted on the clinical data of one EVI1 gene positive child CML-AP patient admitted to Qingdao Women and Children's Hospital in January 2019, and relevant literature was reviewed. The patient, male, 14 years old, was diagnosed and received a combination of domestic imatinib and low-dose chemotherapy, achieving complete hematological remission. The quantification of BCR-ABL p210 decreased to 4.66%, and the quantification of EVI1 decreased to normal (0.01%). Conclusion: CML-AP in children is rare, and its clinical features mainly include a significant increase in white blood cell count and splenomegaly. The combination of domestic imatinib and low-dose chemotherapy is effective in the treatment of CML-AP in children with EVI1 gene positivity, but the long-term efficacy still needs close follow-up.