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儿童复发/难治性急性淋巴细胞白血病的风险适应性治疗结果。

Outcome of risk adapted therapy for relapsed/refractory acute lymphoblastic leukemia in children.

机构信息

Department of Pediatric Hematology/Oncology, King Fahd National Center for Children's Cancer, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia.

出版信息

Leuk Lymphoma. 2013 Mar;54(3):547-54. doi: 10.3109/10428194.2012.719616. Epub 2012 Oct 18.

DOI:10.3109/10428194.2012.719616
PMID:22938053
Abstract

Results of second-line therapy for childhood acute lymphoblastic leukemia (ALL) remain suboptimal, particularly for high-risk groups identified using timing and site of relapse. We report results of prospectively collected data for pediatric patients with ALL who received risk adjusted second-line therapy. The 59 patients who failed first-line ALL therapy included 36 (61%) with bone marrow (BM), 13 (22.1%) with isolated extramedullary (EM) and 10 (16.9%) with BM + EM relapse. Some 51.8% patients were reinduced with high dose cytosine arabinoside (HDAraC)-based and 48.2% with standard four-drug regimens. In all, 38/56 (67.9%) achieved a complete remission (CR) with second-line therapy; the overall CR rate was 78.6% and was not associated with CR1 duration (p =0.8). Three-year overall survival (OS) was 45.3%, and was 61.4% for those achieving a CR. No risk group benefited from HSCT over chemotherapy. Patients with isolated EM relapse beyond 18 months of CR1 and BM relapse beyond 12 months off-therapy had an excellent outcome (OS 91.7%), identifying a particularly good-risk cohort. Patients not in this category continue with poor outcome even following hematopoietic stem cell transplant.

摘要

二线治疗儿童急性淋巴细胞白血病(ALL)的结果仍不理想,尤其是对于通过复发时间和部位确定的高危人群。我们报告了接受风险调整二线治疗的 ALL 儿科患者的前瞻性收集数据结果。59 例一线 ALL 治疗失败的患者中,36 例(61%)为骨髓(BM)复发,13 例(22.1%)为孤立性髓外(EM)复发,10 例(16.9%)为 BM+EM 复发。约 51.8%的患者采用高剂量阿糖胞苷(HDAraC)为基础的再诱导治疗,48.2%采用标准四药方案。共有 56 例中的 38 例(67.9%)在二线治疗中获得完全缓解(CR);总 CR 率为 78.6%,与 CR1 持续时间无关(p=0.8)。3 年总生存率(OS)为 45.3%,CR 患者的 OS 为 61.4%。高危组无获益于 HSCT 优于化疗。CR1 后 18 个月以上出现孤立性 EM 复发和停药后 12 个月以上出现 BM 复发的患者具有极好的结局(OS 91.7%),确定了一个特别低危的队列。不在这一范畴的患者即使接受造血干细胞移植,预后仍较差。

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