摘要
目的分析朗格罕细胞组织细胞增生症（LCH）病例资料，探讨其疗效，分析复发相关因素。方法45例LCH患儿按上海市儿童医院一朗格罕细胞组织细胞增生症-2007方案（SCH—LCH-2007方案）（即改良版DAL～HX83／90方案）分为3组：A组（仅有骨骼病变）18例；B组（任何软组织受累，有或无骨损害）6例；C组（出现脏器受累）21例。分组治疗并阶段性评估。（1）初始方案：A组[醋酸泼尼松（Pred）＋长春新碱（VCR）]疗程28周，B组[Pred＋VCR十足叶乙甙（VPl6）＋6巯基嘌呤（6MP）]疗程43周，C组[Pred＋VCR＋VPl6＋6MP＋甲氨蝶呤（MTX）]疗程52周。（2）复发后再治疗方案：①原方案依次逐步升级治疗（A组升B组、B组升c组）；②c组患者个体方案，如VPl6增减、胸腺肽和／或环孢素维持等。结果45例患儿总有效率达93．3％（42／45例），总复发率为26．7％（12／45例），其中A组和B组患儿全部有效，C组患儿中2例死亡、1例失访。多因素分析发现年龄，性别，组别，骨骼、软组织、皮疹、淋巴结、肺、口腔、耳、垂体受累这些因素对于复发差异无统计学意义；而肝、脾受累和造血功能这3个因素对于是否复发差异有统计学意义（肝P：0．0071、脾P=0．0169、造血功能P=0．0111）。结论儿童LCH病变可多系统受累并反复，改良DAL—HX83／90方案通过分组化疗，总体疗效较好；肝、脾和造血功能受累与复发有相关性。
Objective To analyze the clinical data of children with Langerhans cell histiocytosis （LCH）, to discuss the therapeutic effect, and to analyze the factors related to prognosis. Methods A total of 45 children diag- nosed as LCH were divided into group A （18 cases with bone lesion only） , group B （6 cases with soft tissue lesion）, and group C （21 cases with viscera lesion） according to Shanghai Children＇ s Hospital - LCH - 2007 scheme [ SCH - LCH - 2007 （ modified DAL - HX83/90 ） scheme ]. （ 1 ） Initial treatment ： group A was treated with Prednisone （Pred） ＋ Vincristine （VCR） for 28 weeks,and group B was treated with Pred ＋ VCR ＋ Etoposide （VP16） ＋ Mercaptopurine （6MP） for 43 weeks,and group C was treated with Pred ＋ VCR ＋ VP16 ＋ Methotrexate （MTX） ＋ 6MP for 52 weeks. （2） Re- treatment scheme after relapse included： （~upgrading treatment, group A to group B, group B to group C. （~Individual treatment for group C included VP16 modification, and maintained Thymosin and/or Ciclosporin etc. Results The total survival rate was 93.3 % （42/45 cases） and recurrence rate was 26.7% （ 12/45 cases）. Children in group A and B were all effective, while 2 patients in group C died, and 1 case missed follow - up. Multi - factor analy- sis showed that the factors like age, sex, group, skeleton, soft tissue, erythra,lymph gland, lung, mouth, ears, hypophysis pituitary had no statistical significance, but liver, spleen and blood involvement had statistical significance in disease relapse：liver （P=O. 007 1）,spleen （P=0.016 9）,and blood （P=O. 011 1）. Conclusion LCH can affect several organs of children and relapse, and modified DAL- HX83/90 scheme is very effective. The liver, spleen and hemato- poiesis system involvement is correlates with the relapse.
出处
《中华实用儿科临床杂志》
CSCD
北大核心
2017年第15期1145-1148,共4页
Chinese Journal of Applied Clinical Pediatrics
