CAJ | 학술논문

目的探讨儿童难治、复发朗格汉斯细胞组织细胞增生症(LCH)的挽救治疗方法.方法分析2012年6月北京大学第一医院儿科血液肿瘤病房收治的1例序贯应用数种化疗方案后多次复发的LCH患儿在应用克拉屈滨后病情得到控制的病例,并进行相关文献复习.结果患儿男,生后3个月开始发病,评估为多系统(皮肤、多发骨骼、肺、肝脏等)伴危险器官受累,先后给予LCH-Ⅲ-Group Ⅰ、JLSG-96、DAL-HX90多种化疗方案,缓解后复发3次以上,直至6岁时给予克拉屈滨治疗,剂量为10 mg/(m2·d),连续应用4d,每28天为一疗程,第3～5个疗程联合应用阿糖胞苷[1g/(m2·d)],总疗程5个月,治疗1个疗程后病情缓解,治疗结束后病情完全控制,停药观察8个月病情仍稳定.以“克拉屈滨”“朗格汉斯细胞组织细胞增生症”和“难治性”为关键词,对中国期刊全文数据库(CNKI)、万方数据知识服务平台及生物医学文献检索数据库(PubMed)收录的论文进行检索,11篇文献研究显示克拉屈滨联合或不联合阿糖胞苷治疗难治性LCH,有效率为44％～ 100％,良好反应率22％～ 86％,剂量多为5～13 mg/(m2·d).克拉屈滨单药应用的不良反应主要为血液学毒性和感染,联合应用阿糖胞苷则毒副作用更加明显.结论常规化疗药物对部分难治、复发LCH患儿的疗效有限时,可考虑应用克拉屈滨作为挽救治疗.
목적 탐토인동난치、복발랑격한사세포조직세포증생증(LCH)적만구치료방법.방법 분석2012년6월북경대학제일의원인과혈액종류병방수치적1례서관응용수충화료방안후다차복발적LCH환인재응용극랍굴빈후병정득도공제적병례,병진행상관문헌복습.결과 환인남,생후3개월개시발병,평고위다계통(피부、다발골격、폐、간장등)반위험기관수루,선후급여LCH-Ⅲ-Group Ⅰ、JLSG-96、DAL-HX90다충화료방안,완해후복발3차이상,직지6세시급여극랍굴빈치료,제량위10 mg/(m2·d),련속응용4d,매28천위일료정,제3～5개료정연합응용아당포감[1g/(m2·d)],총료정5개월,치료1개료정후병정완해,치료결속후병정완전공제,정약관찰8개월병정잉은정.이“극랍굴빈”“랑격한사세포조직세포증생증”화“난치성”위관건사,대중국기간전문수거고(CNKI)、만방수거지식복무평태급생물의학문헌검색수거고(PubMed)수록적논문진행검색,11편문헌연구현시극랍굴빈연합혹불연합아당포감치료난치성LCH,유효솔위44％～ 100％,량호반응솔22％～ 86％,제량다위5～13 mg/(m2·d).극랍굴빈단약응용적불량반응주요위혈액학독성화감염,연합응용아당포감칙독부작용경가명현.결론 상규화료약물대부분난치、복발LCH환인적료효유한시,가고필응용극랍굴빈작위만구치료.
Objective To investigate the salvage therapy for a child with refractory and (or) repeatedly-relapsed Langerhans cell histiocytosis.Method Data of a patient with Langerhans cell histiocytosis whose disease relapsed repeatedly treated with cladribine was collected and analyzed and the related literature was reviewed.Result The initial symptoms developed 3 months after his birth,multiple systems (skin,skeleton,lung,liver) were involved;he was sequentially treated with LCH-Ⅲ-Group Ⅰ,JLSG-96,DAL-HX90 chemotherapeutic regimens.The patient got relapses for more than 3 times,but the disease got completely controlled after being treated with cladribine when the patient was 6 years old.The dosage was 10 mg/(m2 · d) for 4 days,and one course lasted for 28 days,the third to fifth courses of treatment used Ara-c in combination,the whole treating time lasted for 5 months.The patient remained in persistent remission for 8 months since discontinuation of treatment." Langerhans cell histiocytosis refractory" " cladribine" were used as the key words to search in the data bases CNKI,Wanfangdata and Pubmed,11 articles were picked.According to the literature,the effective rate of cladribine in treatment of repeatedly relapsing Langerhans cell histiocytosis was 44％-100％,with a good response of 22％-86％,the dose was 5-13 mg/(m2 · d).The main side effects were hematological system damages and infection.Conclusion The effect of commonly used chemotherapeutic regimens is limited for children with refractory and(or) repeatedly-relapsed Langerhans cell histiocytosis and cladribine can be used as an alternative therapeutic option of the salvage therapy.