Preleukemia in children.

Preleukemia in children.
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儿童白血病前期。

DOI:
10.1016/s0022-3476(81)80761-5
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发表时间:
1981
期刊:
The Journal of pediatrics
影响因子:
--
通讯作者:
Lange,B
Lange,B
中科院分区:
--
文献类型:
--
作者:
Blank,J;Lange,B

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6例急性粒单核细胞白血病患儿表现为白血病前期综合征。急性非淋巴细胞白血病患儿中AMML的白血病前期表现的发生率为17%,占该机构所有急性白血病患儿的2.9%,与成人的发生率相似。在白血病前期(3~35个月),所有儿童都出现贫血,感染并发症,6个孩子中有3个有出血倾向。3例在AMML诊断前接受了类固醇治疗,并且都有一定的客观反应。2例患者在接受AMML治疗前死亡。4名接受AMML治疗的儿童,包括阿糖胞苷和蒽环类药物的联合治疗,获得完全缓解。最终,所有患者死亡。白血病前期诊断的线索包括不明原因的血细胞减少(无论是绝对的还是功能性的)、外周原始细胞、进行性巨母细胞增多伴B12值升高、红细胞生成障碍、核分裂异常、非随机染色体改变和体外骨髓集落与簇比率降低。直到有一个非常有效的治疗急性非淋巴细胞白血病,确切地说,当治疗儿童与白血病前期仍然不确定。但是,应在感染性并发症或出血倾向危及生命之前开始治疗。
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 cystosine 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 B12value, 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.
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