a Department of Pathology , University of Colorado School of Medicine , Aurora , CO , USA.
b Department of Pathology , Duke University , Durham , NC , USA.
Leuk Lymphoma. 2019 Apr;60(4):1006-1013. doi: 10.1080/10428194.2018.1508667. Epub 2018 Sep 6.
Acute lymphoblastic leukemia (ALL) in infants <1-year-old is biologically different from ALL in older children. Although KMT2A rearrangement is the predominant genetic signature in infantile B-ALL, disease course is heterogenous, behaving more aggressively in younger infants. We investigated clinicopathological differences throughout the first year to understand the transition to pediatric B-ALL. In a multi-institutional review involving four medical institutions, 54 cases of infantile B-ALL were identified. Patients were divided into congenital and non-congenital groups with multiple age subgroups. Male predominance was seen in congenital cases compared to female in non-congenital cases. There were decreasing trends of hyperleukocytosis, central nervous system involvement, KMT2A rearrangements, lineage switch, and mortality, versus increasing trends of CD10 expression and non-KMT2A abnormalities. Statistically significant differences emerged at 3 and 9 months, the latter was not previously described. Poor-prognostic risk factors decreased with age, the last trimester of infantile B-ALL essentially merging with pediatric B-ALL.
婴儿(<1 岁)急性淋巴细胞白血病(ALL)在生物学上与大龄儿童的 ALL 不同。虽然 KMT2A 重排是婴儿 B-ALL 的主要遗传特征,但疾病过程具有异质性,在较小的婴儿中表现更为侵袭性。我们研究了整个第一年的临床病理差异,以了解向儿科 B-ALL 的转变。在涉及四个医疗机构的多机构审查中,确定了 54 例婴儿 B-ALL。患者分为先天性和非先天性组,并分为多个年龄亚组。与非先天性病例中的女性相比,先天性病例中男性占优势。与 KMT2A 重排、谱系转换和死亡率呈下降趋势相比,白细胞增多、中枢神经系统受累、CD10 表达和非 KMT2A 异常呈上升趋势。3 个月和 9 个月时出现了统计学显著差异,后者以前没有描述过。不良预后危险因素随年龄而减少,婴儿 B-ALL 的最后一个三个月基本上与儿科 B-ALL 合并。