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儿童淋巴细胞白血病的法美英(FAB)形态学分类及其临床重要性。

French American British (FAB) morphological classification of childhood lymphoblastic leukaemia and its clinical importance.

作者信息

Lilleyman J S, Hann I M, Stevens R F, Eden O B, Richards S M

出版信息

J Clin Pathol. 1986 Sep;39(9):998-1002. doi: 10.1136/jcp.39.9.998.

Abstract

As part of the Medical Research Council Leukaemia Trial UKALL VIII, 738 unselected children with acute lymphoblastic leukaemia (ALL) had the morphology of their marrow blast cells reviewed by a panel of three haematologists. Ninety four (13%) showed appearances classifiable as type L2 by the French American and British (FAB) cooperative group's criteria, five (0.7%) were typed L3, and the remaining 639 (86%) as L1. Disregarding the patients classified as L3, those with the L2 variant showed an inferior disease free survival to that of the remainder (p less than 0.01), and more of them failed to remit after receiving "standard" remission induction treatment (p less than 0.01). They included an excess of older children (p less than 0.01) with less profound marrow failure at diagnosis, and fewer of them expressed the common ALL antigen (p = 0.05). There was no association between L2 morphology and the diagnostic white cell count, sex, or the presence of a mediastinal mass. These findings confirm earlier reports that FAB L2 ALL is associated with a poor prognosis and that it occurs more commonly in older children. The high remission failure rate is a recent observation and indicates that alternative early treatment may be appropriate for such patients.

摘要

作为医学研究委员会英国ALL VIII白血病试验的一部分,738名未经挑选的急性淋巴细胞白血病(ALL)患儿的骨髓原始细胞形态由三名血液学家组成的小组进行了评估。根据法美英(FAB)协作组的标准,94名(13%)患儿的细胞形态可分类为L2型,5名(0.7%)为L3型,其余639名(86%)为L1型。不考虑分类为L3型的患者,L2变异型患者的无病生存率低于其余患者(p<0.01),并且他们中更多人在接受“标准”缓解诱导治疗后未缓解(p<0.01)。他们中年龄较大的儿童比例过高(p<0.01),诊断时骨髓衰竭程度较轻,且表达常见ALL抗原的人数较少(p = 0.05)。L2形态与诊断时的白细胞计数、性别或纵隔肿块的存在之间没有关联。这些发现证实了早期报告,即FAB L2 ALL与预后不良相关,且在年龄较大的儿童中更常见。高缓解失败率是最近的一项观察结果,表明对此类患者可能适合采用替代的早期治疗方法。

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