ISSN 1674-3865  CN 21-1569/R
主管：国家卫生健康委员会
主办：中国医师协会
　　　辽宁省基础医学研究所
　　　辽宁中医药大学附属医院
中国中西医结合儿科学

中国中西医结合儿科学 ›› 2017, Vol. 9 ›› Issue (6): 530-532.doi: 10.3969/j.issn.1674-3865.2017.06.023

• 临床研究 • 上一篇    下一篇

儿童朗格罕斯细胞组织细胞增生症临床分析

杨海霞，郑敏翠，李婉丽   

  1. 410007 长沙，湖南省儿童医院血液内科
  • 出版日期:2017-12-25 发布日期:2018-11-19
  • 作者简介:杨海霞（1973-），女，医学硕士，副主任医师。研究方向：小儿血液内科疾病的诊治

Clinical analysis of Langerhans cell histiocytosis in children

YANG Haixia，ZHENG Mincui，LI Wanli   

  1.  Department of Hematology, Hunan Children 's Hospital，Changsha 410007，China
  • Online:2017-12-25 Published:2018-11-19

PDF (PC)

87

摘要：
目的
探讨儿童朗格罕斯细胞组织细胞增生症（LCH）的临床特点。
方法
选取2010年1月至2015年12月湖南省儿童医院血液内科收治的LCH患儿68例的临床资料。根据病史、体格检查、血常规、肝肾功能检查、影像学检查及骨髓细胞形态学检查等进行器官功能评价,将所有病例分为3组。Ⅰ组(多系统高危险度)32例，Ⅱ组(多系统低危险度)20例，Ⅲ组（单系统多发骨损害和局限性特殊部位累及）16例。65例确诊LCH患儿均采取LCH-Ⅲ治疗方案。3例LCH患儿确诊后未进行化疗治疗，其中有1例为皮肤受累的新生儿LCH，2例确诊为锁骨孤立病变，采取病灶刮除术。分析其临床特点、治疗方法及随访结果。
结果
68例LCH患儿中多表现为皮疹、骨损害、肝脾肿大等症状。65例接受化疗，6周总有效率为78.46%（51/65），12个月总有效率为83.08%（54/65）；随访至2016年12月，Ⅰ组痊愈率为58.06%（18/31），Ⅱ组痊愈率为60.00%（12/20），Ⅲ组痊愈率为71.43%（10/14）。
结论
LCH患儿的临床表现各异，病变分布较广，常伴有多器官功能受累，因此，根据临床特征进行分组治疗，可改善其预后，但对多脏器功能损害的LCH患儿，其疗效有待提高。

关键词: 朗格罕斯细胞组织细胞增生症, 临床特点, 儿童

Abstract:
Objective
To investigate the clinical features of Langerhans cell histiocytosis(LCH) in children.
Methods
The clinical data of 68 LCH children admitted to Department of Hematology, Hunan Children's Hospital from January 2010 to December 2015 were selected. According to medical history, physical examination, blood routine, liver and kidney function tests, imaging examination and bone marrow cell morphological examination, organ function was evaluated; all cases were divided into three groups. There were 32 cases in group Ⅰ (multi-system high risk), 20 cases in group Ⅱ (multi-system low-risk), and 16 cases in group Ⅲ (single system with multiple bone lesions and limited special parts involved). Totally 65 cases of confirmed LCH children were given LCH-Ⅲ treatment. Three cases of LCH were not given without chemotherapy after diagnosis, among which 1 was neonatal LCH with skin involved, and 2 cases were single lesion of clavicle and treated by focal curettage. Analyze its clinical features, treatment and follow-up results.
Results
In the 68 cases of LCH rash, bone damage, hepatosplenomegaly and other symptoms were more common. Sixty-five patients received chemotherapy, and the total effective rate was 78.46% (51/65) at 6 weeks, and 83.08% (54/65) at 12 months. The follow-up lasted until December 2016, and the cure rate was 58.06% in group Ⅰ,60.00% in group Ⅱ, and 71.43% in group Ⅲ.
Conclusion
LCH children have different clinical manifestations, lesions are widely distributed and are often accompanied by multiple organ dysfunction; therefore, grouped treatment according to clinical characteristics can improve their prognosis, but for those with multiple organ dysfunction, the efficacy needs to be improved.

Key words: Langerhans cell histiocytosis, Clinical features, Children