机构：[1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing 100045, China医技科室职能科室临床流行病与循证医学中心血液中心首都医科大学附属北京儿童医院[2]Hematology and Oncology Laboratory, Beijing Pediatric Research Institute, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing 100045, China科研平台职能科室临床流行病与循证医学中心血液疾病研究室儿科研究所首都医科大学附属北京儿童医院
Background: Pulmonary Langerhans cell histiocytosis (PLCH) is an interstitial primary pulmonary disease, characterized by Langerhans cell proliferation. It is easily misdiagnosed in children. This study aimed to characterize the clinical manifestations and features of PLCH by retrospective analysis. Methods: A retrospective analysis was performed in 117 PLCH patients out of 338 LCH patients who were admitted in our center from November 2006 to October 2013. Variables between two groups were compared by Mann-Whitney U-test and Chi-square test. Kaplan-Meier curves were constructed to compare the survival rates and Cox regression to evaluate the effect of risk factors. Results: The median age of PLCH group was significantly lower than that of non-PLCH group (18.63 months vs. 43.4 months, P < 0.001). All PLCH children had other organ involvement and only 11 cases (9.4%) had respiratory symptoms. The most common radiologic finding was cystic lesions (29 cases, 24.8%). Pulmonary function abnormalities were dominated by obstructive ventilatory dysfunction (63 cases, 82.9%). The 5-year overall survival (OS) of PLCH children was 93.6% +/- 2.3% and the event-free survival (EFS) was 55.7% +/- 5.2%. Among the 38 cases with progressed or relapsed disease, five cases (13.2%) were due to progression or recurrence of lung damage. The 5-year OS of PLCH children with "risk organ" involvement was significantly lower than those without "risk organ" involvement (86.0% +/- 4.9% vs. 100%, chi(2) = 8.793, P=0.003). The difference of EFS between two groups was also significant (43.7% +/- 7.7% vs. 66.3% +/- 6.5%, chi(2) = 5.399, P = 0.020). The "risk organ" involvement had a significant impact on survival (hazard ratio = 1.9, P = 0.039). Conclusions: PLCH mainly occurs in young children, and only a small percentage of patients have respiratory symptoms. They generally have other organ involvement. Most of PLCH children have a good prognosis and most lung lesions could have improved or stabilized. Management of "risk organ" involvement is the key point to improving EFS.
基金：
National Science and Technology Key Projects [2017ZX09304029004]; Beijing Municipal Science and Technology CommissionBeijing Municipal Science & Technology Commission [Z171100001017050]; National Natural Science Foundation of ChinaNational Natural Science Foundation of China [81700186]; Scientific Research Common Program of Beijing Municipal Commission of EducationBeijing Municipal Commission of Education [KM201710025019]; Pediatric Project of Ai You Foundation [AYEK201802]; National Natural Science Foundation of Beijing Children's Hospital, Capital Medical University [GPY201713]
第一作者机构：[1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing 100045, China
共同第一作者：
通讯作者：
通讯机构：[1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Beijing 100045, China[*1]Beijing Key Laboratory of Pediatric Hematology Oncology, National Key Discipline of Pediatrics (Capital Medical University), Key Laboratory of Major Diseases in Children, Ministry of Education, Hematology Oncology Center, Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Nanlishi Road No. 56, Xicheng District, Beijing 100045, China
推荐引用方式(GB/T 7714)：
Wang Dong,Cui Lei,Li Zhi-Gang,et al.Clinical Research of Pulmonary Langerhans Cell Histiocytosis in Children[J].CHINESE MEDICAL JOURNAL.2018,131(15):-.doi:10.4103/0366-6999.237400.
APA：
Wang, Dong,Cui, Lei,Li, Zhi-Gang,Zhang, Li,Lian, Hong-Yun...&Zhang, Rui.(2018).Clinical Research of Pulmonary Langerhans Cell Histiocytosis in Children.CHINESE MEDICAL JOURNAL,131,(15)
MLA：
Wang, Dong,et al."Clinical Research of Pulmonary Langerhans Cell Histiocytosis in Children".CHINESE MEDICAL JOURNAL 131..15(2018):-
