摘要
目的 分析儿童间变性大细胞淋巴瘤(ALCL)的临床特点及预后相关因素,总结疗效和药物相关毒性.方法 2003年1月至2010年4月北京儿童医院收治的ALCL患儿38例,采用北京儿童医院-ALCL-2003方案.结果 间变性淋巴瘤激酶(ALK)+ 34例,男:女=2.16∶1,中位发病年龄9岁,33例(86.8%)患儿有B症状,94.7%的患儿确诊时已达Ⅲ~Ⅳ期.全组中位随访时间48个月(12 ~ 99个月),中位无病生存时间43个月.34例(89.5%)获得完全缓解.复发3例,均在开始治疗的20个月内发生.预计4年无病生存率为(81.2±6.4)%,预计4年总生存率为(86.4±5.7)%.单因素分析预后不良因素包括:3个以上结外器官受累,肝脾肿大(>3 cm),乳酸脱氢酶升高,临床分期为Ⅳ期,骨髓中出现噬血现象,年龄<3岁.主要药物相关毒性是骨髓抑制和黏膜炎,无化疗相关死亡发生.结论 儿童ALCL易出现结外侵犯,多有B症状.本组患儿化疗效果良好,方案安全性良好,多为早期复发,长春花碱(VBL)维持治疗是必要的.
Objective To analyze the clinical features and prognostic factors of children's anaplastic large cell lymphoma (ALCL),summarize the therapeutic effect and toxicities.Method A total of 38 ALCL patients admitted to Beijing Children's Hospital from Jan.2003 to Apr.2010 were treated with BCH-ALCL-2003 regimen (modified from HK-ALCL-2000).Result Thirty-four cases were ALK +,male:female ratio =2.16∶ 1.The median age was 9 years; 86.8% had B symptoms.94.7% evolved to Stage Ⅲand Ⅳ on admission.The median follow-up duration was 48 months ( 12 to 99 months).Median event-free survival (EFS) time was 43 months.Thirty-four patients (89.5% ) achieved a remission.The disease relapsed in 3 patients within 20 months after diagnosis. Estimated 4-year EFS was ( 81.2 ± 6.4)%,estimated 4-year overall survival (OS) rate was ( 86.4 ± 5.7 ) %.Univariate analysis indicated that the unfavorable prognostic factors included:more than 3 extra nodal involvement,hepatosplenomegaly ( 〉 3cm),elevated lactate dehydrogase ( LDH ),stage Ⅳ,hemophagocytosis in bone marrow,and age 〈 3 years.The major toxicity was myelosuppression and mucositis.no chemotherapy related death occurred.Conclusion (1) Childhood ALCL patients often have B symptoms and extranodal involvement. (2) In the study,therapeutic effects was good.The disease relapsed mostly within the first 2 years,maintenance therapy with vinblastinl is necessary.(3)The regimen is safe to patients.
出处
《中华儿科杂志》
CAS
CSCD
北大核心
2012年第3期223-226,共4页
Chinese Journal of Pediatrics
基金
北京市卫生局首都医学发展科研基金(2007-1030)
关键词
淋巴瘤
大细胞
间变性
治疗
临床研究性
预后
儿童
Lymphoma, large-cell, anaplastic
Therapies, investigational
Prognosis
Child