{"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":"31 1","pages":"847-849"},"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\":\"31 1\",\"pages\":\"847-849\"},\"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.
期刊介绍：
"Cancer Research and Clinic" is a series of magazines of the Chinese Medical Association under the supervision of the National Health Commission and sponsored by the Chinese Medical Association.
It mainly reflects scientific research results and academic trends in the field of malignant tumors. The main columns include monographs, guidelines and consensus, standards and norms, treatises, short treatises, survey reports, reviews, clinical pathology (case) discussions, case reports, etc. The readers are middle- and senior-level medical staff engaged in basic research and clinical work on malignant tumors.
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