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79例t(8;21)急性髓系白血病患儿的预后及染色体异常情况

[Prognosis and chromosomal abnormalities in 79 children with t (8;21) acute myeloid leukemia].

作者信息

Chen Yu-Mei, Liu Tian-Feng, Ruan Min, Zou Yao, Chen Xiao-Juan, Guo Ye, Wang Shu-Chun, Zhu Xiao-Fan

机构信息

Diagnosis and Treatment Center of Pediatric Blood Diseases, Institute of Hematology and Blood Diseases Hospital, CAMS and PUMC, Tianjin 300020, China.

出版信息

Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2009 Oct;31(5):542-6.

Abstract

OBJECTIVE

To investigate the chromosomal abnormalities and evaluate the prognostic value of post-remission chemotherapy in children with t (8;21) acute myeloid leukemia (AML).

METHODS

The diagnosis of AML and its subtyping were performed using morphological, immunological, and cytogenetic methodologies in 79 children. Induction therapies included homoharringtonine and cytarabine (HA), daunorubicin and cytarabine (DA), or homoharringtonine and daunorubicin and cytarabine (HAD). Allogeneic stem cell transplantation or 5-6 cycles of intensive chemotherapy was performed after remission therapy.

RESULTS

Additional chromosomal abnormalities, including loss of sex chromosome (n = 40, 50.6%), del (9q) (n = 9, 11.4%), and complex abnormality (n = 7, 8.9%) were identified in 55 patients (69.6%). Three patients had more than 90 chromosomes and duplicate t (8;21) tetraploid karyotype, and their prognoses were poor. The complete remission (CR) rates were 81.7% (49/60) and 94.8% (55/58), respectively, after one and two cycles of induction chemotherapy. The 3-year event-free survival rate (EFS), disease-free survival rate (DFS), and overall survival rate (OS) were (26.2 +/- 6.8)%, (31.3 +/- 6.7)%, and (27.6 +/- 6.6)%, respectively. Twenty-nine patients received 5 or more cycles of chemotherapy after CR and demonstrated an improved 3-year DFS [(51.7 +/- 9.3)%]. The 3-year DFS was not significantly differently in patients with or without additional abnormalities other than sex chromosome (P = 0.36). Post-remission consolidation by high dose cytarabine (HDAC) was significantly superior to standard chemotherapy (66.7% vs. 27.3%, P = 0.03).

CONCLUSION

Most children with t (8;21) AML have additional chromosomal abnormalities, although they do not affect the prognosis and long-term survival. Few patients have more than 90 chromosomes and duplicate t (8;21) tetraploid karyotype, which may result in poor prognosis. Childhood t (8;21) AML usually has high CR rate with relatively good prognosis, and post-remission consolidation by HDAC can improve the survival.

摘要

目的

探讨t(8;21)急性髓系白血病(AML)患儿的染色体异常情况,并评估缓解后化疗的预后价值。

方法

采用形态学、免疫学和细胞遗传学方法对79例患儿进行AML诊断及其亚型分型。诱导治疗包括高三尖杉酯碱和阿糖胞苷(HA)、柔红霉素和阿糖胞苷(DA)或高三尖杉酯碱、柔红霉素和阿糖胞苷(HAD)。缓解治疗后进行异基因干细胞移植或5 - 6周期的强化化疗。

结果

55例患者(69.6%)发现有额外的染色体异常,包括性染色体丢失(n = 40,50.6%)、9号染色体长臂缺失(n = 9,11.4%)和复杂异常(n = 7,8.9%)。3例患者有超过90条染色体及重复的t(8;21)四倍体核型,其预后较差。诱导化疗1个周期和2个周期后的完全缓解(CR)率分别为81.7%(49/60)和94.8%(55/58)。3年无事件生存率(EFS)、无病生存率(DFS)和总生存率(OS)分别为(26.2±6.8)%、(31.3±6.7)%和(27.6±6.6)%。29例患者在CR后接受了5个或更多周期的化疗,其3年DFS有所改善[(51.7±9.3)%]。除性染色体外有无其他异常的患者3年DFS无显著差异(P = 0.36)。高剂量阿糖胞苷(HDAC)缓解后巩固治疗明显优于标准化疗(66.7%对27.3%,P = 0.03)。

结论

大多数t(8;21)AML患儿有额外的染色体异常,尽管它们不影响预后和长期生存。少数患者有超过90条染色体及重复的t(8;21)四倍体核型,这可能导致预后不良。儿童t(8;21)AML通常CR率高,预后相对较好,HDAC缓解后巩固治疗可提高生存率。

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