144例婴儿白血病临床资料分析
目的 探讨婴儿白血病在临床表现、生物学特征、治疗及预后等方面的特点。方法 回顾性分析1993年8月~2014年10月在我院诊断的婴儿白血病的临床资料并进行文献复习。结果(1)1993年8月~2014年10月期间,共诊断婴儿白血病144例,其中ALL 83例,ML55例(AML 51例,CML 4例),混合细胞白血病1例,细胞学与免疫分型不合5例; (2)年龄分布以9~12月最多,占38.2%;(3)临床表现多样,87.5%(125/144)伴有肝脾肿大,45.5%(46/101)有骨浸润;6例有皮肤浸润,年龄均小于6月(P<0.05);约1/3的病人外周血白细胞总数≥100×109 /L; (4)95例患儿进行了染色体检查,染色体异常者共67例,其中t (4;11)或t (9;11)或t (11;19)阳性者共例18例,年龄越小者,染色体异常的比例越高; (5)37例行了MLL基因检测,其中11例阳性,阳性率约为30%。阳性者有11号染色体异常的比例高于阴性者(P<0.05); (6)诊断后大多数患儿放弃治疗,仅有25例接受过化疗,其中有8例仅完成诱导缓解化疗后就放弃治疗,仅有6例患儿按疗程规范治疗,该6例患儿目前均处于完全缓解期。结论 婴儿白血病为罕见类型白血病,临床特征与其他类型白血病有所不同,容易伴有肝脾肿大,外周血白血病总数升高,MLL融合基因阳性或11号染色体异常。目前婴儿白血病的治疗仍是以化疗为主,造血干细胞移植治疗较化疗没有明显的优势,也有关于婴儿白血病的临床试验研究。由于各种原因,本文中规范治疗的婴儿白血病例数很少,对于婴儿白血病的疗效及预后评估仍需扩大样本量进行随访及关注。
Objective To explore the clinical features, laboratory findings and treatment of infant leukemia. Methods A retrospective analysis of the clinical data was performed of the cases with the diagnosis of infant acute leukemia from August 1993 to October 2014 in our hospital. Results A total of 144 cases of infant leukemia were diagnosed in the defined period, including 83 cases of acute lymphoblastic leukemia, 55 myeloid leukemia, 1 hybrid acute leukaemia and 5 with incompatible cytological and immunophenotyping findings. The patients at the age of 9 to 12 months accounted for the largest proportion (38.2%), and 87.5% of the patients had hepatosplenomegaly; Six patients below 6 months old had skin infiltration. In about 1/3 of the patients, the white blood cells count was no greater than 10009 /L. Ninety-five patients had chromosome examinations, which identified chromosome abnormalities in 67 patients, including 18 positive for t(4;11)or t(9;11)or t(11;19), and younger patients were more likely to have chromosome abnormalities. Thirty-seven patients underwent MLL gene detection and 11 of them had positive results; the positive patients had higher rate of chromosome 11 abnormalities than the negative patients. Most of the patients gave up treatments after diagnosis and only 6 patients older than 6 months completed regular chemotherapeutic treatments and were now in complete remission. Conclusion Infant leukemia is a rare type of leukemia with different clinical features from other types of leukemia. The patients often present with hepatosplenomegaly, high white blood cell counts, MLL gene fusion, and chromosome 11 abnormalities. The prognosis of infant leukemia is not favorable, and the current treatment still relies on chemotherapy.
肖剑文、窦颖、于洁、宪莹、徐酉华、温贤浩、管贤敏、郭玉霞
临床医学儿科学肿瘤学
婴儿白血病MLL基因治疗
肖剑文,窦颖,于洁,宪莹,徐酉华,温贤浩,管贤敏,郭玉霞.144例婴儿白血病临床资料分析[EB/OL].(2017-12-07)[2025-07-16].https://chinaxiv.org/abs/201712.00699.点此复制
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