Teyssier Anne-Charlotte, Lapillonne Hélène, Pasquet Marlene, Ballerini Paola, Baruchel André, Ducassou Stephane, Fenneteau Odile, Petit Arnaud, Cuccuini Wendy, Ragu Christine, Preudhomme Claude, Mercher Thomas, Sirvent Nicolas, Leverger Guy
a Department of Pediatric Onco-Hematology , University Hospital Arnaud de Villeneuve , Montpellier , France.
b Hematology Laboratory, Armand Trousseau Hospital, APHP , Paris , France.
Pediatr Hematol Oncol. 2017 Nov;34(8):425-427. doi: 10.1080/08880018.2017.1414905. Epub 2018 Jan 5.
We report the outcome of 27 children with de novo acute megakaryoblastic leukemia (AMKL) (excluding Down syndrome) enrolled in the French multicenter prospective study ELAM02 (2005-2011). There was no difference in gender, initial leukocyte count, CNS involvement, and complete remission rate (88.9%), as compared to other acute myeloid leukemia (AML) subtypes. AMKL patients had a significantly poorer outcome (5-year overall survival 54% [CI 95% 33%-71%] than children with other AML subtypes (5-year overall survival 73% [CI 95% 68%-77%] p = 0.02). Gender, age, CNS leukemia, hyperleukocytosis, complete remission or cytogenetic subgroups were not significant prognostic factors of disease-free survival. AMKL (excluding Down syndrom) remains an AML subgroup with inferior outcome.
我们报告了27例初发急性巨核细胞白血病（AMKL）（不包括唐氏综合征）患儿的研究结果，这些患儿参加了法国多中心前瞻性研究ELAM02（2005 - 2011年）。与其他急性髓系白血病（AML）亚型相比，在性别、初始白细胞计数、中枢神经系统受累情况及完全缓解率（88.9%）方面并无差异。AMKL患儿的预后明显较差（5年总生存率为54% [95%置信区间33% - 71%]），低于其他AML亚型患儿（5年总生存率为73% [95%置信区间68% - 77%]，p = 0.02）。性别、年龄、中枢神经系统白血病、白细胞增多症、完全缓解或细胞遗传学亚组并非无病生存的显著预后因素。（不包括唐氏综合征的）AMKL仍然是一个预后较差的AML亚组。
