Blank J, Lange B
J Pediatr. 1981 Apr;98(4):565-8. doi: 10.1016/s0022-3476(81)80761-5.
Six children who developed acute myelomonocytic leukemia presented with a preleukemic syndrome. The incidence of preleukemic presentation of AMML was 17% of children with acute nonlymphocytic leukemia, or 2.9% of all children with acute leukemia at this institution, incidences similar to those in adults. During the preleukemic phase, which lasted from three to 35 months, all children had anemia, all had infectious complications, and three of six had hemorrhagic tendencies. Three received steroids before the diagnosis of AMML, and all had some objective response. Two patients died before receiving therapy for AMML. Four children who received AMML therapy with combinations including cytosine arabinoside and an anthracycline achieved complete remission. Ultimately, all patients died. Clues to diagnosis of preleukemia include unexplained cytopenias, either absolute or functional, peripheral blasts, progressive megaloblastosis with an elevated B12 value, dyserythropoiesis, abnormalities of nuclear segmentation, nonrandom chromosomal alterations, and reduced marrow colony to cluster ratio in vitro. Until there is a highly effective therapy for ANLL, precisely when to treat the child with preleukemia remains uncertain. However, treatment should be started before infectious complications or hemorrhagic tendencies become life-threatening.
6例发生急性粒单核细胞白血病的儿童出现了白血病前期综合征。急性粒单核细胞白血病白血病前期表现的发生率在该机构为急性非淋巴细胞白血病患儿的17%，或所有急性白血病患儿的2.9%，这一发生率与成人相似。在持续3至35个月的白血病前期阶段，所有儿童均有贫血，均有感染并发症，6例中有3例有出血倾向。3例在急性粒单核细胞白血病诊断前接受了类固醇治疗，且均有一定的客观反应。2例患者在接受急性粒单核细胞白血病治疗前死亡。4例接受包括阿糖胞苷和蒽环类药物联合治疗的急性粒单核细胞白血病患儿获得完全缓解。最终，所有患者均死亡。白血病前期诊断线索包括不明原因的血细胞减少（绝对或功能性）、外周血原始细胞、B12值升高的进行性巨幼细胞贫血、红细胞生成异常、核分叶异常、非随机染色体改变以及体外骨髓集落与集簇比例降低。在有针对急性非淋巴细胞白血病的高效治疗方法之前，究竟何时治疗白血病前期儿童仍不确定。然而，应在感染并发症或出血倾向危及生命之前开始治疗。
