小児コア結合因子急性骨髄性白血病の臨床的特徴と予後

[Clinical features and prognosis of core binding factor acute myeloid leukemia in children].

• Chao Liu
• Xiao-Yan Chen
• Mei-Hui Yi
• Wen-Qi Wu
• Min Ruan
• Xiao-Fan Zhu

抄録

目的:

本研究では、コア結合因子を有する急性骨髄性白血病（CBF-AML）の小児における臨床的特徴と予後を検討した。

OBJECTIVE: To study the clinical features and prognosis of core binding factor acute myeloid leukemia (CBF-AML) in children.

方法:

METHODS: A retrospective analysis was performed from the chart review data of children who were newly diagnosed with CBF-AML in the Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences, from August 2009 to November 2015. According to the type of fusion gene, the children were divided into CBFB-MYH11 and AML1-ETO groups. Clinical features and prognosis were analyzed and compared between the two groups.

結果:

CBF-AML患者91名が登録され、そのうちAML1-ETO群74名（81％）、CBFB-MYH11群17名（19％）が登録された。追加の染色体異常は38人（42％）に認められ、性染色体欠失が最も多く28人（31％）に認められた。導入治療の初回コース後の完全寛解率は97％（88/91）、再発率は29％（26/91）、5年イベントフリー生存（EFS）率は65％±6％、5年全生存（OS）率は75％±5％であった。5年EFS率（62％±7％ vs 77％±11％、P＞0.05）、5年OS率（72％±6％ vs 88％±9％、P＞0.05）については、AML1-ETO群とCBFB-MYH11群の間に有意差はなかった。

RESULTS: A total of 91 children with CBF-AML were enrolled in this study, among whom there were 74 (81%) in the AML1-ETO group and 17 (19%) in the CBFB-MYH11 group. Additional chromosomal abnormalities were observed in 38 children (42%), and deletion of sex chromosome was the most common abnormality and was observed in 28 children (31%). After the first course of induction treatment, the complete remission rate was 97% (88/91), the recurrence rate was 29% (26/91), the 5-year event-free survival (EFS) rate was 65%±6%, and the 5-year overall survival (OS) rate was 75%±5%. There were no significant differences between the AML1-ETO and CBFB-MYH11 groups in 5-year EFS rate (62%±7% vs 77%±11%, P>0.05) or 5-year OS rate (72%±6% vs 88%±9%, P>0.05).

結論:

CBF-AMLの小児ではAML1-ETOが主な融合遺伝子であり、性染色体の欠失は追加染色体異常の中で最も多いタイプです。CBF-AMLの小児は予後が良好なことが多く、AML1-ETOを有する小児はCBFB-MYH11と同様の予後を示します。

CONCLUSIONS: AML1-ETO is the main type of fusion gene in children with CBF-AML, and deletion of sex chromosome is the most common type of additional chromosomal abnormalities. Children with CBF-AML often have a good prognosis, and the children with AML1-ETO have a similar prognosis to those with CBFB-MYH11.