微小残留病值可区分21号染色体存在染色体内扩增的B细胞前体急性淋巴细胞白血病患儿的低复发风险和高复发风险：奥地利及德国急性淋巴细胞白血病柏林-法兰克福-明斯特（ALL-BFM）试验
Minimal residual disease values discriminate between low and high relapse risk in children with B-cell precursor acute lymphoblastic leukemia and an intrachromosomal amplification of chromosome 21: the Austrian and German acute lymphoblastic leukemia Berlin-Frankfurt-Munster (ALL-BFM) trials.
作者信息
Attarbaschi Andishe, Mann Georg, Panzer-Grümayer Renate, Röttgers Silja, Steiner Manuel, König Margit, Csinady Eva, Dworzak Michael N, Seidel Markus, Janousek Dasa, Möricke Anja, Reichelt Carsten, Harbott Jochen, Schrappe Martin, Gadner Helmut, Haas Oskar A
机构信息
Department of Pediatric Hematology and Oncology, St Anna Children's Hospital, Vienna, Austria.
出版信息
J Clin Oncol. 2008 Jun 20;26(18):3046-50. doi: 10.1200/JCO.2008.16.1117.
PURPOSE
We aimed to identify relapse predictors in children with a B-cell precursor acute lymphoblastic leukemia (ALL) and an intrachromosomal amplification of chromosome 21 (iAMP21), a novel genetic entity associated with poor outcome.
PATIENTS AND METHODS
We screened 1,625 patients who were enrolled onto the Austrian and German ALL-Berlin-Frankfurt-Münster (ALL-BFM) trials 86, 90, 95, and 2000 with ETV6/RUNX1-specific fluorescent in situ hybridization probes, and we identified 29 patient cases (2%) who had an iAMP21. Minimal residual disease (MRD) was quantified with clone-specific immunoglobulin and T-cell receptor gene rearrangements.
RESULTS
Twenty-five patients were good responders to prednisone, and all achieved remission after induction therapy. Eleven patients experienced relapse, which included eight who experienced relapse after cessation of front-line therapy. Six-year event-free and overall survival rates were 37% +/- 14% and 66% +/- 11%, respectively. Results of MRD analysis were available in 24 (83%) of 29 patients: nine (37.5%) belonged to the low-risk, 14 (58.5%) to the intermediate-risk, and one (4%) to the high-risk group. MRD results were available in 8 of 11 patients who experienced a relapse. Seven occurred among the 14 intermediate-risk patients, and one occurred in the high-risk patient.
CONCLUSION
The overall and early relapse rates in the BFM study were lower than that in a previous United Kingdom Medical Research Council/Childhood Leukemia Working Party study (38% v 61% and 27% v 47%, respectively), which might result from more intensive induction and early reintensification therapy in the ALL-BFM protocols. MRD values were the only reliable parameter to discriminate between a low and high risk of relapse (P = .02).
目的
我们旨在确定B细胞前体急性淋巴细胞白血病（ALL）伴21号染色体内部扩增（iAMP21）患儿的复发预测因素，iAMP21是一种与不良预后相关的新的遗传实体。
患者与方法
我们用ETV6/RUNX1特异性荧光原位杂交探针筛查了1625例纳入奥地利和德国ALL-Berlin-Frankfurt-Münster（ALL-BFM）86、90、95和2000试验的患者，确定了29例（2%）患有iAMP21的患者病例。用克隆特异性免疫球蛋白和T细胞受体基因重排对微小残留病（MRD）进行定量分析。
结果
25例患者对泼尼松反应良好，诱导治疗后均获得缓解。11例患者复发，其中8例在一线治疗结束后复发。6年无事件生存率和总生存率分别为37%±14%和66%±11%。29例患者中有24例（83%）可获得MRD分析结果：9例（37.5%）属于低风险组，14例（58.5%）属于中风险组，1例（4%）属于高风险组。11例复发患者中有8例可获得MRD结果。7例发生在14例中风险患者中，1例发生在高风险患者中。
结论
BFM研究中的总体复发率和早期复发率低于英国医学研究委员会/儿童白血病工作组先前的一项研究（分别为38%对61%和27%对47%），这可能是由于ALL-BFM方案中诱导治疗和早期强化治疗更加强烈。MRD值是区分低复发风险和高复发风险的唯一可靠参数（P = 0.02）。
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