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台湾地区婴儿白血病的临床特征、分子诊断和治疗结果。

Clinical features, molecular diagnosis, and treatment outcome of infants with leukemia in Taiwan.

机构信息

Division of Hematology/Oncology, Department of Pediatrics, Chang Gung Memorial Hospital, Taoyuan, Taiwan.

出版信息

Pediatr Blood Cancer. 2010 Dec 15;55(7):1264-71. doi: 10.1002/pbc.22731.

DOI:10.1002/pbc.22731
PMID:20979094
Abstract

BACKGROUND

Infant leukemia is rare and quite distinct from other childhood leukemias. Differentiating between leukemia and transient myeloproliferative disorder (TMD) in phenotypically normal infants is sometimes difficult. The clinical features and molecular analyses for the fusion transcripts of mixed lineage leukemia (MLL) gene rearrangement in infant leukemia have not been well documented in the Chinese population.

PROCEDURE

Forty-five consecutive infants diagnosed with leukemia between 1995 and 2007 in a tertiary medical center in Taiwan were studied. Acute lymphoblastic leukemia (ALL) was diagnosed in 23 infants, acute myeloid leukemia (AML) in 21 (including TMD in 4), and juvenile myelomonocytic leukemia (JMML) in 1.

RESULTS

The median white count at diagnosis was higher in ALL than in AML (154.4 × 10(9)/l vs. 58.3 × 10(9)/l, P = 0.05). Chromosome 11q23/MLL abnormalities were present in 77% of ALL and 31% of AML. The 5-year event-free survival (EFS) in infant ALL and AML showed no difference (18% vs. 12%, respectively). The only independent predictor of an adverse prognosis among infants diagnosed with ALL was high presenting white count ≥ 100 × 10(9)/l (P = 0.05). However, no factor was associated with an adverse outcome for infants with AML.

CONCLUSIONS

The molecular assessments and prognostic factors of infant leukemia in Taiwan mirror those in developed Western countries. Continued molecular investigations and development of more effective therapies are needed.

摘要

背景

婴儿白血病较为罕见,与其他儿童白血病明显不同。在表型正常的婴儿中,区分白血病和短暂性髓系增生异常(TMD)有时较为困难。在中国人中,混合谱系白血病(MLL)基因重排融合转录本的婴儿白血病的临床特征和分子分析尚未得到很好的记录。

方法

在台湾的一家三级医疗中心,对 1995 年至 2007 年间连续诊断的 45 例婴儿白血病患者进行了研究。诊断为急性淋巴细胞白血病(ALL)的有 23 例,急性髓细胞白血病(AML)的有 21 例(包括 4 例 TMD),幼年粒单核细胞白血病(JMML)的有 1 例。

结果

ALL 患儿的诊断时中位白细胞计数明显高于 AML(154.4×10^9/L 比 58.3×10^9/L,P=0.05)。ALL 中 77%存在 11q23/MLL 异常,AML 中 31%存在 11q23/MLL 异常。婴儿 ALL 和 AML 的 5 年无事件生存率(EFS)无差异(分别为 18%和 12%)。ALL 患儿中,高白细胞计数(≥100×10^9/L)是不良预后的唯一独立预测因素(P=0.05)。然而,AML 患儿的预后与任何因素均无关。

结论

台湾婴儿白血病的分子评估和预后因素与发达国家相似。需要进一步的分子研究和开发更有效的治疗方法。

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