Department of Hematology Oncology, Children's Hospital & Research Center Oakland, Oakland, CA, USA.
Br J Haematol. 2015 Feb;168(4):533-46. doi: 10.1111/bjh.13160. Epub 2014 Oct 13.
Children and adolescents presenting with a markedly elevated white blood cell (ME WBC) count (WBC ≥200 × 10(9) /l) comprise a unique subset of high-risk patients with acute lymphoblastic leukaemia (ALL). We evaluated the outcomes of the 251 patients (12% of the study population) with ME WBC treated on the Children's Cancer Group-1961 protocol. Patients were evaluated for early response to treatment by bone marrow morphology; those with a rapid early response were randomized to treatment regimens testing longer and stronger post-induction therapy. We found that ME WBC patients have a poorer outcome compared to those patients presenting with a WBC <200 × 10(9) /l (5-year event-free survival 62% vs. 73%, P = 0·0005). Longer duration of therapy worsened outcome for T cell ME WBC with a trend to poorer outcome in B-ALL ME WBC patients. Augmented therapy benefits T cell ME WBC patients, similar to the entire study cohort, however, there appeared to be no impact on survival for B-ALL ME WBC patients. ME WBC was not a prognostic factor for T cell patients. In patients with high risk features, B lineage disease in association with ME WBC has a negative impact on survival.
儿童和青少年表现出明显升高的白细胞计数(WBC≥200×10(9)/l),构成了急性淋巴细胞白血病(ALL)高危患者的一个独特亚组。我们评估了在儿童癌症组 1961 方案中接受治疗的 251 例 ME WBC 患者(占研究人群的 12%)的结局。通过骨髓形态学评估患者对早期治疗的快速反应;那些快速早期反应的患者被随机分配到测试更长和更强诱导后治疗的治疗方案中。我们发现,与 WBC<200×10(9)/l 的患者相比,ME WBC 患者的结局较差(5 年无事件生存率 62% vs. 73%,P=0.0005)。治疗时间延长会使 T 细胞 ME WBC 的结局恶化,B-ALL ME WBC 患者的结局也有恶化趋势。强化治疗对 T 细胞 ME WBC 患者有益,与整个研究队列相似,但对 B-ALL ME WBC 患者的生存没有影响。ME WBC 不是 T 细胞患者的预后因素。在具有高危特征的患者中,B 系疾病与 ME WBC 并存对生存有负面影响。