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目的探讨儿童伴肺部改变的朗格汉斯细胞组织细胞增生症(PLCH)的临床表现、CT特征性表现、诊断及治疗特点,并分析其与预后的关系,为PLCH患者临床方案的制定提供依据。方法回顾性分析本院2008年1月至2014年1月经病理确诊的17例PLCH患儿的临床特征、影像学资料及接受Ⅲ型化疗方案(诱导阶段6周+维持阶段52周)化疗后的疗效和预后。结果 17例PLCH患儿起病时伴有肺部弥漫性改变为主的多脏器损害,在疾病发展过程中,胸部CT表现出特征性变化。17例均接受Ⅲ型方案化疗,其中8例完全缓解,预后良好;4例化疗后复发,其中1例预后良好,3例死亡;3例化疗中疾病进展放弃治疗,死亡;1例化疗中疾病进展再诱导化疗,目前持续化疗中,存活;1例化疗维持治疗中,目前持续随访。结论儿童伴肺部改变的LCH多为婴幼儿起病,伴多脏器损害,早期高强度化疗可改善预后,但部分病例肺部进展明显,预后不良。
Objective To investigate the clinical manifestations, CT features, diagnosis and treatment characteristics of Langerhans cell histiocytosis (PLCH) in children with pulmonary changes, and to analyze the relationship between the characteristics of the diagnosis and treatment of PLCH and to provide a clinical plan for PLCH patients. in accordance with. Methods A retrospective analysis was performed on the clinical features, radiological data, and chemotherapy regimens (17 weeks of induction and 52 weeks of maintenance) in patients with PLCH diagnosed by pathology from January 2008 to January 2014 in our hospital. Efficacy and prognosis. Results Seventeen children with PLCH had multiple organ damages that were mainly diffuse pulmonary changes at the onset. During the course of disease development, chest CT showed characteristic changes. All the 17 patients received type III regimen chemotherapy, 8 of whom had complete remission and a good prognosis. Four patients had relapse after chemotherapy. One patient had a good prognosis and 3 patients died. Three patients died of chemotherapy during disease progression and died. One patient experienced chemotherapy disease. Progressive re-induction chemotherapy, current continuous chemotherapy, survival; 1 case of chemotherapy maintenance therapy, ongoing follow-up. Conclusion LCH in children with lung changes is mostly infantile onset, with multiple organ damage. Early high-intensity chemotherapy can improve the prognosis, but in some cases, the lung progress is obvious and the prognosis is poor.