17例伴有肺部改变的朗格汉斯细胞组织细胞增生症的临床观察

Analysis of clinical manifestations and treatment of pulmonary Langerhans cell histiocytosis: A 17 cases report

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 study the characteristic clinical manifestations,CT manifestations,diagnosis and treatment characteristics,and analyze its relationships with prognosis of the children with pulmonary Langerhans cell histiocytosis（ PLCH）. Methods In a retrospective study,the clinical features and imaging data of 17 children with PLCH were analyzed as well as the efficacy and prognosis after type Ⅲ chemotherapy of 6-week induction phase and 52-week maintenance phase. Results All children suffered onset pulmonary diffuse change with multiple organ damage and in the process of disease development; most of the chest computed tomography（ CT） showed characteristic changes. All the 17 children were received type Ⅲ chemotherapy,and 8 cases got complete remission with good prognosis,4 cases relapsed after chemotherapy including 1 case with good prognosis and 3 cases of death,3 cases abandoned treatment due to progression during chemotherapy. In addition,1 case with progression received re-induction chemotherapy and survived with the present continuous chemotherapy. One case of maintenance chemotherapy treatment was followed-up. Conclusion Children with PLCH were infants and young children with multiple organ damage. Early intenstive chemotherapy can improve the prognosis,but some cases of lung progress are apparent.