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儿童混合表型急性白血病15例临床特征分析

Clinical characteristics of 15 children with mixed phenotype acute leukemia

摘要:

目的:分析儿童混合表型急性白血病(MPAL)的临床特点、治疗及预后,为临床优化诊疗方案及提高缓解率提供参考。方法:基于2016年世界卫生组织(WHO)的诊断标准,回顾性分析2012年1月至2020年12月苏州大学附属儿童医院收治的15例MPAL患儿的骨髓细胞形态、免疫分型、细胞遗传学、分子生物学特征以及治疗方案、预后等病例资料。计数数据组间比较采用 χ2检验,符合正态分布的计量资料组间比较采用 t检验,非正态分布的计量资料组间比较采用秩和检验。采用 Kaplan- Meier( K- M)法估计生存率,比较应用 Log- rank法。 结果:苏州大学附属儿童医院8年共收治15例MPAL患儿,男8例,女7例,中位年龄为6.8岁;9例患儿表达B淋系+髓系表型,5例表达T淋系+髓系表型,1例表达B淋系+T淋系表型;11例患儿进行了染色体核型检查,2例为正常核型,2例为复杂核型,6例为假二倍体,1例为亚二倍体;5例患儿检测到融合基因,其中3例 AML- ETO阳性,1例 BCR- ABL阳性,1例 MLL阳性;13例患儿在化疗后完全缓解,总完全缓解率为86.6%,2年总生存率为(68.2±13.4)%。15例患儿中14例授受了诱导化疗,1例因个人原因放弃了治疗。首选急性淋巴细胞白血病(ALL)化疗方案10例,第1个疗程完全缓解1例,总完全缓解率10%;首选急性髓系白血病(AML)化疗方案4例,第1个疗程完全缓解3例,总完全缓解率75%,未缓解的1例更换ALL方案后缓解;8例行造血干细胞移植(HSCT)和6例未行HSCT组2年总生存率分别为(70.0±18.2)%、(66.7±19.2)%,差异无统计学意义( χ2=0.318, P=0.573)。 结论:儿童MPAL是一种罕见的恶性肿瘤,以淋系和髓系抗原共表达为主,单纯化疗或HSCT在短期内均可获得较好的预后,但长期疗效还有待进一步观察。

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abstracts:

Objective:To analyze the clinical characteristics, treatment and prognosis of mixed phenotypic acute leukemia (MPAL) in children, and to provide reference for clinical optimization of diagnosis and treatment and improve the remission rate.Methods:Based on the diagnostic criteria of the World Health Organization (WHO) in 2016, the bone marrow cell morphology, immune typing, cytogenetics, molecular biological characteristics, treatment regimen, and prognosis of 15 children with MPAL who were admitted to Children′s Hospital Affiliated to Soochow University from January 2012 to December 2020 were retrospectively analyzed.The count data were compared between groups using the χ2 test, the measurement data that conformed to the normal distribution were compared using the t test, and the measurement data that were not normally distributed were compared using the rank sum test.Survival was estimated using the Kaplan- Meier ( K- M) method and the Log- rank method was used for comparison. Results:A total of 15 children with MPAL were admitted to Children′s Hospital Affiliated to Soochow University in the past 8 years, including 8 males and 7 females, with a median age of 6.8 years.Nine patients expressed B lymphocyte and myeloid phenotype, 5 patients expressed T lymphocyte and myeloid phenotype, and 1 patient expressed B and T lymphocyte phenotype.Karyotype of 11 children was examined, and the results showed that there were 2 cases of normal karyotype, 2 complex karyotype, 6 pseudodiploid and 1 subdiploid.Fusion genes were detected in 5 children, including 3 AML- ETO positive, 1 BCR- ABL positive, and 1 MLL gene positive.Thirteen patients were in complete remission (CR) after chemotherapy, with a total CR rate of 86.6% and a 2-year over survival (OS) rate of (68.2±13.4)%.Among the 15 children, 14 received induction chemotherapy and 1 gave up treatment for personal reasons.There were 10 patients with the first choice of acute lymphoblastic leukemia (ALL) chemotherapy regimen and 1 patient receiving CR, with a total CR rate of 10%.There were 4 cases of acute myeloid leukemia (AML) with the preferred chemotherapy regimen and 3 cases with CR in the first course of treatment, and the total CR rate was 75%.One case without remission was relieved after changing ALL chemotherapy regimen.The 2-year OS rates of 8 patients with hematopoietic stem cell transplantation (HSCT) and 6 patients without HSCT were (70.0±18.2)% and (66.7±19.2)%, respectively, with no significant difference ( χ2=0.318, P=0.573). Conclusions:Children with MPAL is a rare malignant tumor, mainly characterized by the co-expression of lymphoid and myeloid antigens.Chemotherapy alone or HSCT can achieve a good prognosis in the short term, but the long-term efficacy remains to be further observed.

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作者: 王宇轩 [1] 黄园园 [1] 何海龙 [2]
期刊: 《中华实用儿科临床杂志》2022年37卷11期 837-841页 ISTICPKUCSCD
栏目名称: 血液/肿瘤疾病
DOI: 10.3760/cma.j.cn101070-20210604-0641
发布时间: 2022-07-31
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