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10岁以上儿童青少年急性淋巴细胞白血病的临床特点及预后分析
Clinical characteristics and prognostic analysis of children and adolescents over 10 years of age with acute lymphoblastic leukemia
目的 研究10岁以上儿童青少年初诊急性淋巴细胞白血病（ALL）患儿的临床特点及预后。方法 对86例10岁以上ALL患儿（B-ALL 62例，T-ALL 24例）的临床特点、治疗疗效及预后因素进行回顾性分析，采用Kaplan-Meier分析评估患儿无事件生存率（EFS）和总生存率（OS），COX回归模型评估EFS、OS的影响因素。结果 86例患儿中，中危组和高危组患儿分别为62例和24例。首诊表现为肝肿大的53例（62%），脾肿大50例（58%），淋巴结肿大46例（54%）。初诊时外周血WBC≥50×109/L者29例（34%）。78例进行了染色体核型分析，21例（27%）染色体数目异常，其中15例为超二倍体（19%）、4例（5%）为亚二倍体，2例（3%）为假二倍体；11例（14%）染色体结构异常，其中Ph染色体阳性1例，t（1；19）1例。TEL-AML1融合基因3例（4%），E2A-PBX1融合基因3例（4%），BCR-ABL融合基因6例（7%），SIL-TAL1融合基因4例（5%）。1疗程完全缓解率为99%（85/86）。5年EFS率、OS率分别为64%±6%和75%±5%。中危组的5年EFS率、OS率均高于高危组（P < 0.05）。B-ALL患儿5年EFS率优于T-ALL组（P < 0.05）。COX多因素回归分析显示，初诊时白细胞计数、诱导缓解末微小残留病是EFS、OS的独立影响因素。结论 10岁以上ALL患儿具有不利预后的临床特征，初诊时白细胞计数、诱导缓解末MRD是决定远期疗效的重要因素。
Objective To explore the clinical characteristics and prognosis of children and adolescents over 10 years of age with acute lymphoblastic leukemia (ALL). Methods A total of 86 newly diagnosed ALL children and adolescents over 10 years of age (62 cases of B-ALL and 24 cases of T-ALL) were enrolled. Clinical characteristics, therapeutic effect and prognostic factors were retrospectively analyzed. Event-free survival (EFS) and overall survival (OS) rates were estimated by the Kaplan-Meier method. Prognostic factors were evaluated by COX regression analysis. Results Of 86 patients, 62 were in medium risk, and 24 in high risk. At diagnosis, 53 patients (62%) had hepatomegaly, 50 patients (58%) had splenomegaly, and 46 patients (54%) had lymphoadenopathy. Twenty-nine patients (34%) showed high leukocyte counts (≥ 50×109/L) at diagnosis. The karyotype analysis was performed on 78 patients. The percentage of hyperdiploidy was 19% (15 cases), and that of hypodiploidy was 5% (4 cases). Eleven patients (14%) had abnormalities of chromosome structure. Of them, one patient was Philadelphia chromosome-positive, and another patient had the t (1; 19) chromosomal translocation. Three patients (4%) were positive for TEL/AML1, 3 (4%) were positive for E2A/PBX1, 6 were positive for BCR/ABL (7%), and 4 (5%) were positive for SIL/TAL1. During 4 weeks of induction therapy, 85 patients (99%) achieved complete remission (CR). In 86 patients, the 5-year anticipated EFS and OS were (64±6)% and (75±5)% respectively. The 5-year EFS and OS in the medium risk group were significantly higher than those in the high risk group (P < 0.05). The 5-year EFS in B-ALL patients was significantly higher than that in T-ALL patients (P < 0.05). COX multivariate analysis showed that white blood counts at diagnosis and minimal residual disease (MRD) after induction therapy were independent prognostic factors. Conclusions Children and adolescents with ALL over 10 years of age often have clinical characteristics of unfavorable prognosis. White blood counts at diagnosis and MRD after induction therapy may be important factors for the long-term prognosis.
急性淋巴细胞白血病 / 预后 / 生存分析 / 儿童 / 青少年
Acute lymphoblastic leukemia / Prognosis / Survival analysis / Child / Adolescent
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