摘要
目的分析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。方法回顾性分析新疆维吾尔自治区人民医院皮肤性病科确诊的8例LCH临床资料,结合国内外文献讨论其临床特点、组织病理及诊疗方法。结果3例患儿均为维族男性,LCH累及多个系统和脏器,头皮表现类似脂溢性皮炎,躯干、四肢皮损呈多形性,病情严重。治疗上给予化疗,部分预后不佳。5例成人患者为单一皮肤型LCH,皮损分布局限或累及全身,形态多样,极易误诊,病情相对稳定,但需长期随访观察。结论LCH是一组可累及儿童及成人的少见病,临床表现复杂,皮损无特异性,应尽早行皮损组织病理及免疫组化明确诊断,同时进一步完善实验室及影像学检查。多系统受累与危险器官受累、初诊时血细胞下降、炎性指标升高对LCH病情判断有重要意义。
Objective To investigate the clinical features and therapeutic approaches in order to improve the quality of diagnosis and treatment of Langerhans cell histiocytosis(LCH).Methods The clinical data of 8 cases of LCH diagnosed in People’s Hospital of Xinjiang Uygur Autonomous Region were analyzed retrospectively alone with related literature associated with the characteristics of clinical manifestations,pathological changes,diagnosis and treatment.Results All the 3 children were Uygur males,LCH involved many systems and organs,the scalps were similar to seborrheic dermatitis,the skin lesions on the body were pleomorphic.The conditions were serious.Chemotherapy was taken in the treatment and some patients had poor prognoses.5 cases of adult patients with single skin type LCH.The location of the rash was either local or systemic,and the skin lesions were various.This LCH was easily to be misdiagnosed and the condition was relatively stable,but it needed long-term follow-up.Conclusion LCH is a group of rare diseases affecting adults and children.The clinical manifestations are complex and lesions are non-specific.Skin histopathology and immunohistochemistry should be done as soon as possible in order to diagnose LCH early.At the same time,the laboratory and imaging examination should be further improved.The important significance of LCH condition judgment is multiple systems and/or risk organ involvement,decreased blood cells at initial diagnosis and increased inflammatory indexes.
作者
屈园园
李文政
康晓静
QU Yuan-yuan;LI Wen-zheng;KANG Xiao-jing(Department of Dermatology,People's Hospital of Xinjiang Uygur Autonomous Region,Xinjiang Key Laboratory of Dermatology Research(XJYS1707),Urumchi 830001,China)
出处
《实用皮肤病学杂志》
2022年第2期94-97,共4页
Journal of Practical Dermatology
基金
自治区创新环境(人才、基地)建设专项(人才专项计划-天山创新团队)(202011254)。
