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[慢性髓性白血病急变期儿童的临床特征及预后研究]

[Research on the clinical characteristics and prognosis of children with chronic myeloid leukemia in the blast phase].

作者信息

Zheng F Y, Lu A D, Jia Y P, Zuo Y X, Zeng H M, Jiang Q, Zhang L P

机构信息

Peking University People's Hospital, Department of Pediatrics, Beijing 100044, China.

Peking University People's Hospital, Peking University Institute of Hematology, National Clinical Research Center for Hematologic Disease, Beijing 100044, China.

出版信息

Zhonghua Xue Ye Xue Za Zhi. 2024 Oct 14;45(10):931-936. doi: 10.3760/cma.j.cn121090-20240130-00045.

Abstract

To explore the clinical characteristics and prognosis of children with chronic myeloid leukemia in the blast phase (CML-BP) . The clinical characteristics, treatment measures, and survival outcomes of 28 children with CML-BP were analyzed in our hospital from January 2008 to November 2022. The male to female ratio of the 28 children with CML-BP was 1.15∶1. The median age of diagnosis of CML-BP was 10 years, and the median follow-up time was 79 months. During the diagnosis of CML, four children were in the BP, one was in the accelerated phase (AP) and 23 children were in the chronic phase (CP). Among the 23 children with CML-CP, 75% had progressed directly from CP to BP without experiencing the AP. Among the children diagnosed with CML-BP, 71.4% were classified as chronic myeloid leukemia lymphoid blast phase (CML-LBP), 25.0% belonged to the chronic myeloid leukemia myeloid blast phase (CML-MBP), and 3.6% belonged to the chronic myeloid leukemia mixed phenotype acute leukemia (CML-MPAL). Treatment with hemaopoietic stem cell transplantation (HSCT) after tyosine kinase inhibitor (TKI) combined with chemotherapy was administered to 19 children, two children received HSCT after TKI alone, and seven children received TKI combined with chemotherapy but without HSCT. The 5-year overall survival of the 28 children with CML-BP was 59.3%. The direct progression of BP from CP is greater in children with CML-BP compared with adults, and the overall prognosis of children with CML-BP is poor.

摘要

探讨儿童慢性髓性白血病急变期(CML - BP)的临床特征及预后。分析2008年1月至2022年11月我院收治的28例CML - BP患儿的临床特征、治疗措施及生存结局。28例CML - BP患儿男女比例为1.15∶1。CML - BP诊断时的中位年龄为10岁,中位随访时间为79个月。在CML诊断时,4例患儿处于急变期,1例处于加速期(AP),23例处于慢性期(CP)。在23例CML - CP患儿中,75%直接从CP进展至BP,未经历AP。在诊断为CML - BP的患儿中,71.4%被分类为慢性髓性白血病淋巴细胞急变期(CML - LBP),25.0%属于慢性髓性白血病髓细胞急变期(CML - MBP),3.6%属于慢性髓性白血病混合表型急性白血病(CML - MPAL)。19例患儿在酪氨酸激酶抑制剂(TKI)联合化疗后接受造血干细胞移植(HSCT),2例患儿仅在TKI治疗后接受HSCT,7例患儿接受TKI联合化疗但未进行HSCT。28例CML - BP患儿的5年总生存率为59.3%。与成人相比,CML - BP患儿中BP直接从CP进展的比例更高,且CML - BP患儿的总体预后较差。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e411/11579757/508ccac6417d/cjh-45-10-931-g001.jpg

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