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成人急性髓系白血病患者 21 三体的生物学和临床特征。

Biological and clinical features of trisomy 21 in adult patients with acute myeloid leukemia.

机构信息

Department of Leukemia, University of Texas M.D. Anderson Cancer Center, Houston, TX.

出版信息

Clin Lymphoma Myeloma Leuk. 2013 Sep;13 Suppl 2(0 2):S276-81. doi: 10.1016/j.clml.2013.05.020. Epub 2013 Aug 19.

Abstract

INTRODUCTION

Trisomy 21 is frequently noted in patients with AML. In adults, +21 has traditionally been considered an intermediate-risk cytogenetic aberration.

PATIENTS AND METHODS

We analyzed 90 patients with newly diagnosed AML harboring +21. Four cytogenetic subgroups were defined based on associated cytogenetic abnormalities: +21 alone, +21 with favorable, +21 with intermediate, and +21 with unfavorable cytogenetics.

RESULTS

Fifty-four percent of patients with +21 AML achieved a complete remission (CR) or CR with incomplete platelet recovery (CRp) after induction therapy with a trend toward improved CR/CRp rates in patients with +21 alone/+21 with favorable cytogenetics compared with patients with +21 with intermediate/+21 with unfavorable cytogenetics (76% vs. 50%; P = .057). Time to progression (TTP) was 12 months (range, 5-19) and overall survival (OS) was 9 months (range, 7-11) for the entire group. TTP was longer for patients with +21 alone (not reached) or with +21 with favorable cytogenetics (101 months) compared with those with +21 with intermediate cytogenetics (2 months) or +21 with unfavorable cytogenetics (11 months) (P = .02). Similarly, OS was improved in patients with +21 with favorable cytogenetics (not reached) or +21 alone (107 months), compared with +21 with unfavorable cytogenetics (9 months) or +21 with intermediate cytogenetics (8 months) (P < .001). The differences in TTP and OS were maintained on multivariate analysis (P = .04 and P = .001; respectively).

CONCLUSION

Isolated +21 hitherto classified as intermediate-risk cytogenetics might actually behave as a favorable-risk cytogenetics in adult AML patients.

摘要

介绍

唐氏综合征 21 三体在 AML 患者中经常被发现。在成年人中,+21 传统上被认为是一种中等风险的细胞遗传学异常。

患者和方法

我们分析了 90 例新诊断为 AML 且携带+21 的患者。根据相关细胞遗传学异常,将这 90 例患者分为四个细胞遗传学亚组:单纯+21、+21 伴有利细胞遗传学、+21 伴中等细胞遗传学和+21 伴不利细胞遗传学。

结果

在诱导治疗后,有 54%的+21 AML 患者达到完全缓解(CR)或不完全血小板恢复的完全缓解(CRp),与+21 伴中等/+21 伴不利细胞遗传学患者相比,单纯+21/+21 伴有利细胞遗传学患者的 CR/CRp 率有改善趋势(76% vs. 50%;P =.057)。整个队列的无进展生存期(TTP)为 12 个月(范围为 5-19),总生存期(OS)为 9 个月(范围为 7-11)。与+21 伴中等细胞遗传学(2 个月)或+21 伴不利细胞遗传学(11 个月)患者相比,单纯+21 或+21 伴有利细胞遗传学患者的 TTP 更长(未达到)(P =.02)。同样,与+21 伴不利细胞遗传学(9 个月)或+21 伴中等细胞遗传学(8 个月)患者相比,+21 伴有利细胞遗传学(未达到)或单纯+21 患者的 OS 得到改善(P <.001)。多变量分析也证实了 TTP 和 OS 的差异(P =.04 和 P =.001)。

结论

以前被归类为中等风险细胞遗传学的单纯+21 实际上可能在成年 AML 患者中表现为有利风险细胞遗传学。

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