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双表型CD2+CD19+急性淋巴细胞白血病患儿的临床特征及治疗结果:一项儿童癌症研究组的研究

Clinical features and treatment outcome of children with biphenotypic CD2+ CD19+ acute lymphoblastic leukemia: a Children's Cancer Group study.

作者信息

Uckun F M, Gaynon P, Sather H, Arthur D, Trigg M, Tubergen D, Nachman J, Steinherz P, Sensel M G, Reaman G R

机构信息

Biotherapy Institute, University of Minnesota, Roseville 55113, USA.

出版信息

Blood. 1997 Apr 1;89(7):2488-93.

PMID:9116293
Abstract

Leukemic cells from a subset of children with acute lymphoblastic leukemia (ALL) express lymphoid antigens of both T lineage and B lineage, but the clinical significance of this immunophenotype is unknown. We now report the first comprehensive comparison of treatment outcomes among a large cohort of children with CD2+ CD19+ biphenotypic ALL (N = 77), B-lineage ALL (BL) (N = 1,631), or T-lineage ALL (TL) (N = 347) ALL who were treated on risk-adjusted Children's Cancer Group (CCG) protocols. CD2+ CD19+ patients were more similar to BL than TL patients with respect to presenting features and antigen expression. The percentages of patients achieving successful induction therapy outcome were 98.7%, 97.8%, and 97.3% for CD2+ CD19+, BL, and TL patients, respectively. Univariate comparisons of 4-year event-free survival (83.7%, 72.8%, 75.2% for CD2+ CD19+, BL, and TL patients, respectively) achieved borderline significance (CD2+ CD19+ B, P = .08; CD2+ CD19+ v T, P = .07). Relative hazard rate (RHR) estimates for BL and TL compared with CD2+ CD19+ were 1.79 and 1.90, respectively, implying a better outcome for biphenotypic patients. However, multivariate adjusted RHRs for BL and TL compared with CD2+ CD19+ were 1.43 (P = .29) and 1.16 (P = .76), respectively, suggesting a significant reduction in risk for BL or TL patients once adjustment was made for the more favorable characteristics of the CD2+ CD19+ group. Thus, pediatric ALL patients treated on contemporary CCG protocols who present with CD2+ CD19+ biphenotypic leukemia generally have good treatment outcomes, due in part to their favorable presenting features.

摘要

一部分急性淋巴细胞白血病(ALL)患儿的白血病细胞表达T系和B系的淋巴样抗原,但这种免疫表型的临床意义尚不清楚。我们现在报告了一大群接受风险调整儿童癌症组(CCG)方案治疗的CD2+CD19+双表型ALL(N = 77)、B系ALL(BL)(N = 1,631)或T系ALL(TL)(N = 347)ALL患儿治疗结果的首次全面比较。CD2+CD19+患者在呈现特征和抗原表达方面与BL患者比与TL患者更相似。CD2+CD19+、BL和TL患者成功诱导治疗结果的百分比分别为98.7%、97.8%和97.3%。4年无事件生存率的单变量比较(CD2+CD19+、BL和TL患者分别为83.7%、72.8%、75.2%)达到临界显著性(CD2+CD19+与B,P = 0.08;CD2+CD19+与T,P = 0.07)。与CD2+CD19+相比,BL和TL的相对风险率(RHR)估计分别为1.79和1.90,这意味着双表型患者的预后更好。然而,与CD2+CD19+相比,BL和TL的多变量调整RHR分别为1.43(P = 0.29)和1.16(P = 0.76),这表明一旦对CD2+CD19+组更有利的特征进行调整,BL或TL患者的风险会显著降低。因此,接受当代CCG方案治疗的表现为CD2+CD19+双表型白血病的儿科ALL患者通常有良好的治疗结果,部分原因是他们具有有利的呈现特征。

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