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探讨儿童朗格汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)的临床特点及治疗方法,以提高LCH的临床诊疗水平。方法:28例儿童LCH,确诊前均行组织病理检查,按Lavin-Osband分级和临床分型,I型和I级、Ⅱ级LCH患儿中单一骨损害采用手术刮除,单一皮肤损害、Ⅱ型、Ⅲ型、Ⅲ级、Ⅳ级采用泼尼松诱导化疗和随后长春新碱加泼尼松(VP)持续化疗。结果:LCH可累及多个系统和脏器,伴有不同的功能障碍,5例单一皮肤型LCH因皮疹减轻或消退家属拒绝化疗。7例单发的骨损害采用手术刮除后痊愈,10例单一皮肤型LCH和6例系统型LCH采用联合化疗。6周后总体疗效评价:治愈率46.43%;18个月后总体疗效评价:治愈率82.14%。结论:LCH是一组特异性以骨髓源性朗格汉斯细胞细胞(Langerhans cell,LC)和成熟的嗜酸性粒细胞增生为特征的先天性疾病,表现为骨、皮肤和淋巴结等局部器官或全身的LC的异常增生。LCH主要发生在儿童,成人少见,临床表现多种多样,经免疫组化可以获得正确诊断,但目前临床缺乏理想统一的治疗方案。泼尼松诱导化疗和随后长春新碱加泼尼松(VP)持续化疗治疗儿童LCH的疗效满意,不良反应、并发症少,值得临床推广。
To investigate the clinical features and treatment of Langerhans cell histiocytosis (LCH) in children, in order to improve the clinical diagnosis and treatment of LCH. Methods: Twenty-eight children underwent histopathological examination before diagnosis. According to Lavin-Osband classification and clinical classification, single bone lesion in type I, grade I and grade II children underwent surgical curettage, single skin lesion, Type, type III, grade III, grade IV with prednisone-induced chemotherapy followed by vincristine plus prednisone (VP) continuous chemotherapy. Results: LCH could affect multiple systems and organs with different dysfunction. Five patients with single cutaneous LCH regressed or exacerbated their rashes and refused chemotherapy. Seven cases of single bone lesions were cured by surgical curettage, 10 cases of single skin type LCH and 6 cases of systemic LCH combined chemotherapy. After 6 weeks, the overall effect evaluation: the cure rate was 46.43%; 18 months after the overall effect evaluation: cure rate 82.14%. CONCLUSION: LCH is a group of congenital diseases characterized by bone marrow-derived Langerhans cell (LC) and mature eosinophilia, which are characterized by local organs such as bone, skin and lymph nodes or Abnormal LC of the whole body. LCH occurs mainly in children, rare in adults, a variety of clinical manifestations, by immunohistochemistry can be correctly diagnosed, but the current lack of clinical ideal uniform treatment. Prednisone chemotherapy and subsequent vincristine plus prednisone (VP) continuous chemotherapy in children with LCH satisfactory results, adverse reactions, fewer complications, worthy of clinical promotion.