临床肿瘤学杂志
臨床腫瘤學雜誌
림상종류학잡지
CHINESE CLINICAL ONCOLOGY
2014年
12期
1135-1137
,共3页
陆世丰%方拥军%芮耀耀%何璐璐%周莉%黄婕%吴鹏%林如峰%王永韧%陆勤
陸世豐%方擁軍%芮耀耀%何璐璐%週莉%黃婕%吳鵬%林如峰%王永韌%陸勤
륙세봉%방옹군%예요요%하로로%주리%황첩%오붕%림여봉%왕영인%륙근
儿童%朗格汉斯细胞组织细胞增生症%临床分析
兒童%朗格漢斯細胞組織細胞增生癥%臨床分析
인동%랑격한사세포조직세포증생증%림상분석
Children%Pulmonary Langerhans cell histiocytosis%Clinical analysis
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。
目的：探討兒童伴肺部改變的朗格漢斯細胞組織細胞增生癥（PLCH）的臨床錶現、CT特徵性錶現、診斷及治療特點，併分析其與預後的關繫，為PLCH患者臨床方案的製定提供依據。方法迴顧性分析本院2008年1月至2014年1月經病理確診的17例PLCH患兒的臨床特徵、影像學資料及接受Ⅲ型化療方案（誘導階段6週＋維持階段52週）化療後的療效和預後。結果17例PLCH患兒起病時伴有肺部瀰漫性改變為主的多髒器損害，在疾病髮展過程中，胸部CT錶現齣特徵性變化。17例均接受Ⅲ型方案化療，其中8例完全緩解，預後良好；4例化療後複髮，其中1例預後良好，3例死亡；3例化療中疾病進展放棄治療，死亡；1例化療中疾病進展再誘導化療，目前持續化療中，存活；1例化療維持治療中，目前持續隨訪。結論兒童伴肺部改變的LCH多為嬰幼兒起病，伴多髒器損害，早期高彊度化療可改善預後，但部分病例肺部進展明顯，預後不良。
목적：탐토인동반폐부개변적랑격한사세포조직세포증생증（PLCH）적림상표현、CT특정성표현、진단급치료특점，병분석기여예후적관계，위PLCH환자림상방안적제정제공의거。방법회고성분석본원2008년1월지2014년1월경병리학진적17례PLCH환인적림상특정、영상학자료급접수Ⅲ형화료방안（유도계단6주＋유지계단52주）화료후적료효화예후。결과17례PLCH환인기병시반유폐부미만성개변위주적다장기손해，재질병발전과정중，흉부CT표현출특정성변화。17례균접수Ⅲ형방안화료，기중8례완전완해，예후량호；4례화료후복발，기중1례예후량호，3례사망；3례화료중질병진전방기치료，사망；1례화료중질병진전재유도화료，목전지속화료중，존활；1례화료유지치료중，목전지속수방。결론인동반폐부개변적LCH다위영유인기병，반다장기손해，조기고강도화료가개선예후，단부분병례폐부진전명현，예후불량。
Objective To study the characteristic clinical manifestations, CT manifestations, diagnosis and treatment charac?teristics, 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 prog?nosis after typeⅢchemotherapy of 6?week induction phase and 52?week maintenance phase. Results All children suffered onset pul?monary 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 typeⅢchemotherapy, 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.
