儿童间变性大细胞淋巴瘤38例临床特征及疗效观察

Clinical features and therapeutic effect of 38 children with anaplastic large cell lymphoma

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.