Lampert F, Harbott J, Ritterbach J
Kinderpoliklinik, Justus-Liebig-Universität, Giessen, Germany.
Br J Cancer Suppl. 1992 Aug;18:S20-2.
Of 706 children, 528 with acute lymphoblastic leukaemia (ALL) and 178 with acute myelocytic leukaemia (AML), whose leukaemia karyotypes could be successfully analysed, 48 were infants less than 1 year of age, 28 with ALL (5% of ALL patients) and 20 with AML (11% of AML patients). In contrast to older children. ALL-leukaemocytogenetics in infants was characterised by lack of hyperdiploidy with over 50 chromosomes and higher incidence of pseudodiploidy. Thirteen (= 46%) infants had an 11q23 aberration, and 11 of them had t(4;11). In AML, nine (= 45%) infants also had an 11q23 abnormality, e.g. t(9;11). Thus, the 11q23 aberration was present in almost 50% of all leukaemia karyotypes of infants. In ALL of infants, the CALLA negative, pre-pre-B immunophenotype prevailed. In AML of infants, the monocytic subtype dominated. A biphenotypic morphology (lymphoid-monocytic) with the expression of lymphoid and myeloid antigens was seen in several ALL and AML cases. In conclusion, leukaemogenesis in infants is a rare event, arising in stem cells of very early hematopoietic differentiation (probably due to gene rearrangement errors, most frequently at FRA11B), and differs from leukaemogenesis in older age groups by unique clinical and cellular features.
在706名儿童中,528例患有急性淋巴细胞白血病(ALL),178例患有急性髓细胞白血病(AML),其白血病核型能够成功分析,其中48例为1岁以下婴儿,28例患ALL(占ALL患者的5%),20例患AML(占AML患者的11%)。与大龄儿童不同,婴儿ALL的白血病细胞遗传学特征是缺乏超过50条染色体的超二倍体,假二倍体发生率更高。13名(=46%)婴儿存在11q23畸变,其中11名有t(4;11)。在AML中,9名(=45%)婴儿也有11q23异常,如t(9;11)。因此,11q23畸变存在于几乎50%的婴儿白血病核型中。在婴儿ALL中,CALLA阴性、前前B免疫表型占主导。在婴儿AML中,单核细胞亚型占主导。在几例ALL和AML病例中可见具有淋巴样和髓样抗原表达的双表型形态(淋巴样-单核细胞)。总之,婴儿白血病的发生是一种罕见事件,发生于非常早期造血分化的干细胞中(可能由于基因重排错误,最常见于FRA11B),并且在临床和细胞特征方面不同于大龄儿童组的白血病发生。