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儿童急性淋巴细胞白血病诊断时的高白细胞计数:生物学背景和预后影响。来自 NOPHO ALL-92 和 ALL-2000 研究的结果。

High white blood cell count at diagnosis of childhood acute lymphoblastic leukaemia: biological background and prognostic impact. Results from the NOPHO ALL-92 and ALL-2000 studies.

机构信息

Centre for Paediatric Oncology and Haematology, University Childrens Hospital, Vilnius, Lithuania.

出版信息

Eur J Haematol. 2011 Jan;86(1):38-46. doi: 10.1111/j.1600-0609.2010.01522.x. Epub 2010 Nov 15.

DOI:10.1111/j.1600-0609.2010.01522.x
PMID:21077959
Abstract

Prognostic impact of peripheral blood white blood cell count (WBC) at the diagnosis of childhood acute lymphoblastic leukaemia (ALL) was evaluated in a population-based consecutive series of 2666 children aged 1-15 treated for ALL between 1992 and 2008 in the five Nordic countries (Denmark, Finland, Iceland, Norway and Sweden). Ten-year event-free (pEFS(10 y)) survival and overall (pOS(10 y)) survival were 0.75 ± 0.01 and 0.85 ± 0.01, respectively. Although treatment intensity was determined by WBC, non-remission and relapsed patients still had significantly higher WBC than those in remission for B-cell precursor (BCP) (median WBC: 24.8 vs. 14.0 vs. 8.3 × 10(9) /L, P < 0.001), but not for T-lineage (T-ALL) (median WBC: 127.8 vs. 113.0 vs. 86.8 × 10(9) /L, P = 0.22). pEFS was inversely related to WBC for BCP (P < 0.001), but not for T-ALL. WBC was not associated with risk of event for BCP or T-ALL for patients with minimal residual disease at the end of induction (MRD(d29) ) <10(-3). In contrast, for MRD(d29) ≥ 10(-3) and <5% leukaemic blasts in bone marrow at day 29, the pEFS(5 y) for WBC < 100.0 (N = 152) vs. ≥ 100.0 (N = 19) was 0.76 vs. 0.50 (P = 0.001). That was the case both for BCP (pEFS(5 y) 0.76 vs. 0.58) and for T-ALL (pEFS(5 y) 0.71 vs. 0.38). Whether the inferior EFS for the subset of patients with high WBC and slow initial response to treatment reflects rare or overlooked cytogenetic aberrations as well as the factors that determine WBC levels at diagnosis awaits exploration.

摘要

在基于人群的连续系列中,评估了外周血白细胞计数(WBC)在诊断儿童急性淋巴细胞白血病(ALL)时的预后影响,该系列包括 1992 年至 2008 年间在五个北欧国家(丹麦、芬兰、冰岛、挪威和瑞典)治疗的 2666 名年龄在 1-15 岁的 ALL 儿童。10 年无事件生存(pEFS(10 y))和总生存(pOS(10 y))分别为 0.75 ± 0.01 和 0.85 ± 0.01。尽管治疗强度由 WBC 决定,但未缓解和复发患者的 WBC 仍明显高于缓解患者的 WBC,无论是 B 细胞前体(BCP)(中位数 WBC:24.8 vs. 14.0 vs. 8.3 × 10(9)/L,P < 0.001),还是 T 细胞谱系(T-ALL)(中位数 WBC:127.8 vs. 113.0 vs. 86.8 × 10(9)/L,P = 0.22)。pEFS 与 BCP 中的 WBC 呈负相关(P < 0.001),但与 T-ALL 无关。对于诱导结束时微小残留病(MRD(d29))<10(-3)的患者,WBC 与 BCP 或 T-ALL 的事件风险无关。相反,对于 MRD(d29)≥10(-3)且 29 天骨髓中白血病细胞<5%的患者,WBC<100.0(N = 152)与≥100.0(N = 19)的 pEFS(5 y)分别为 0.76 和 0.50(P = 0.001)。对于 BCP(pEFS(5 y)0.76 vs. 0.58)和 T-ALL(pEFS(5 y)0.71 vs. 0.38),情况均如此。对于 WBC 较高且对治疗初始反应较慢的患者亚组的不良 EFS 是否反映了罕见或被忽视的细胞遗传学异常以及决定诊断时 WBC 水平的因素,有待进一步探讨。

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