CAJ | 학술논문

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.
목적 분석인동간변성대세포림파류(ALCL)적림상특점급예후상관인소,총결료효화약물상관독성.방법 2003년1월지2010년4월북경인동의원수치적ALCL환인38례,채용북경인동의원-ALCL-2003방안.결과 간변성림파류격매(ALK)+ 34례,남:녀=2.16∶1,중위발병년령9세,33례(86.8％)환인유B증상,94.7％적환인학진시이체Ⅲ～Ⅳ기.전조중위수방시간48개월(12 ～ 99개월),중위무병생존시간43개월.34례(89.5％)획득완전완해.복발3례,균재개시치료적20개월내발생.예계4년무병생존솔위(81.2±6.4)％,예계4년총생존솔위(86.4±5.7)％.단인소분석예후불량인소포괄:3개이상결외기관수루,간비종대(＞3 cm),유산탈경매승고,림상분기위Ⅳ기,골수중출현서혈현상,년령＜3세.주요약물상관독성시골수억제화점막염,무화료상관사망발생.결론 인동ALCL역출현결외침범,다유B증상.본조환인화료효과량호,방안안전성량호,다위조기복발,장춘화감(VBL)유지치료시필요적.
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.