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诊断时的髓外浸润对急性髓细胞白血病患儿无预后意义。

Extramedullary infiltrates at diagnosis have no prognostic significance in children with acute myeloid leukaemia.

作者信息

Bisschop M M, Révész T, Bierings M, van Weerden J F, van Wering E R, Hählen K, van der Does-van den Berg A

机构信息

Department of Paediatric Haematology-Oncology, University Medical Centre, Utrecht, The Netherlands.

出版信息

Leukemia. 2001 Jan;15(1):46-9. doi: 10.1038/sj.leu.2401971.

Abstract

This retrospective study was designed to review the relative frequency and prognostic significance of extramedullary infiltrates in children with acute myeloid leukaemia (AML). The registration data and initial discharge summaries were reviewed for all children diagnosed with AML, and registered by the Dutch Childhood Leukaemia Study Group (DCLSG). Between 1972 and 1998, 477 children were diagnosed with AML. Of these patients, 120 (25.1%) had extramedullary leukaemia (EML) at diagnosis. Four categories of EML were found: skin, soft tissue or bone, gingival infiltration and central nervous system (CNS) involvement. Patients who presented with gingival infiltrates, were older than those without EML or those in the other EML subgroups, had a high initial WBC count and a high proportion of M4/M5 morphological variants. This type of presentation could indicate a special biological entity. Univariate analysis of prognostic factors in patients treated after 1985 with intensive protocols showed that initial WBC count and the presence of favourable cytogenetic findings were significant. The presence of EML at diagnosis had no significant effect on event-free survival. In a stepwise multiple regression analysis only favourable cytogenetic findings remained significant.

摘要

这项回顾性研究旨在评估急性髓系白血病(AML)患儿髓外浸润的相对发生率及其预后意义。荷兰儿童白血病研究组(DCLSG)对所有诊断为AML并登记的儿童的登记数据和初次出院小结进行了回顾。1972年至1998年间,477名儿童被诊断为AML。其中,120例(25.1%)在诊断时患有髓外白血病(EML)。发现了四类EML:皮肤、软组织或骨骼、牙龈浸润和中枢神经系统(CNS)受累。出现牙龈浸润的患者比无EML或其他EML亚组的患者年龄大,初始白细胞计数高,M4/M5形态学变异比例高。这种表现类型可能表明存在一种特殊的生物学实体。对1985年后采用强化方案治疗的患者的预后因素进行单因素分析显示,初始白细胞计数和有利的细胞遗传学结果具有显著意义。诊断时EML的存在对无事件生存期无显著影响。在逐步多元回归分析中,只有有利的细胞遗传学结果仍然具有显著意义。

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