CAJ | 학술논문

目的：总结和探讨朗格罕细胞组织细胞增生症（langerhans cell histiocytosis，LCH）合并恶性肿瘤的类型、发病机制、两者之间的关系及治疗。方法对我院收治的2例朗格罕细胞组织细胞增生症合并恶性肿瘤患儿的病历资料进行回顾性总结并复习相关文献。结果2例患儿均为LCH合并神经母细胞瘤，两病同时诊断，患儿年龄均＜2岁，联合化疗及手术切除治疗预后良好。国外文献报道在大部分LCH和淋巴瘤、肺癌相关的病例中，LCH发生在确诊恶性肿瘤后的2年内，且和原发恶性肿瘤有紧密的病理联系，表明瘤细胞可诱发朗格罕细胞增生。而大多数LCH-白血病和LCH-其它实体瘤患者恶性肿瘤发生在LCH诊断后的较长时间，化疗继发恶性肿瘤可能性大。本文中报导的2例两种疾病同时诊断，它们之间的相互关系尚不明确。结论 LCH合并恶性肿瘤临床比较罕见， LCH可能是特殊的树突细胞对恶性肿瘤反应增生的结果，或恶性肿瘤继发于LCH化疗，或者是两者独立存在，应以治疗恶性肿瘤为主，可兼顾LCH化疗。
목적：총결화탐토랑격한세포조직세포증생증（langerhans cell histiocytosis，LCH）합병악성종류적류형、발병궤제、량자지간적관계급치료。방법대아원수치적2례랑격한세포조직세포증생증합병악성종류환인적병력자료진행회고성총결병복습상관문헌。결과2례환인균위LCH합병신경모세포류，량병동시진단，환인년령균＜2세，연합화료급수술절제치료예후량호。국외문헌보도재대부분LCH화림파류、폐암상관적병례중，LCH발생재학진악성종류후적2년내，차화원발악성종류유긴밀적병리련계，표명류세포가유발랑격한세포증생。이대다수LCH-백혈병화LCH-기타실체류환자악성종류발생재LCH진단후적교장시간，화료계발악성종류가능성대。본문중보도적2례량충질병동시진단，타문지간적상호관계상불명학。결론 LCH합병악성종류림상비교한견， LCH가능시특수적수돌세포대악성종류반응증생적결과，혹악성종류계발우LCH화료，혹자시량자독립존재，응이치료악성종류위주，가겸고LCH화료。
Objective Summarize and discuss the type,pathogenesis and treatment of Langerhans cell histiocytosis (LCH ) accompanied with cancer,as well as relationship between LCH and cancer. Method Retrospectively summarize 2 cases of LCH accompanied with cancer treated in our hospital and review the relevant literatures.Result 2 patients treated in our hospital are children younger than 2 years old.Both of them are LCH accompanied with neuroblastoma which happened at the same time.Prognosis of two patients are well for chemotherapy and surgical resection are conducted concurrently.Foreign literature reported 9 1 cases of LCH accompanied with cancer and divided them into 3 groups:1 .LCH accompanied with malignant lymphoma;2.LCH accompanied with Leukemia;3.LCH accompanied with solid tumor.In most related cases of LCH accompanied with lymphoma or lung cancer,diagnosis of LCH usually happened within 2 years after diagnosis of cancer,and LCH had close pathological connection with primary cancer, which indicated Langerhans cell proliferation might be the reaction to tumor cells.While great majority of LCH accompanied with lymphoma and other solid tumor had cancer occurrence long after diagnosis of LCH, while possibility of cancer secondary to cancer is greater.2 patients in our study have two diseases happened at the same time,but relationship between them was still unknown.Conclusion It is rare of LCH accompanied with cancer clinically,LCH might be result of sorts of specific dendritic cell reactively proliferation to cancer,or cancer secondary to LCH chemotherapy,or each disease exists independently. Treatment should be given priority to cancer and given consideration to LCH chemotherapy.