Clinical And Pulmonary Imaging Analysis Of Langerhans Cell Histiocytosis In Children

Objective: To discuss the clinical and lung CT images of LCH in children,and to analyze the relationship of the clinical characteristics with the CT manifestations,for understanding the disease well in diagnosis.Methods: A retrospective study was performed on the 140 patients diagnosed LCH at the Children’s Hospital of Chongqing Medical University between January 1,2008 to December 31,2015,79 cases were male and 61 were female,median age of onset were range 0 from 15.4 years old. According clinical classification and lesions,divide LCH into SS-LCH and MS-LCH,according lungs involved or not,divide MS-LCH into PLCH and lungs uninvolved MS-LCH. According the age,involved organs/systems,risk organs divide LCH into 4 classification. Analysising the clinical and CT characteristics,comparing the relevance between clinical characteristics and lungs CT manifestations.Results:1.There were 63 patients in SS-LCH,48 patients(75%) had symptoms after 2 years old,58 patients(90.6%) were skeleton involved alone;76 patients in MS-LCH,46 patients(60.5%)disease onset in less than 2 years old,many organs/systems could involved,the most common involoved organ was liver(59/76,77.6%),followed by skin(57/76,75.0%) and spleen(49/76,64.5%).There were 33 cases in PLCH,29 cases(87.9%)involve skin,43 cases children who lungs uninvolved,28 cases(65.1%) involve skin,there was significant difference between two groups(P<0.05).2. The ratio of clinical grading in SS-LCH was 25.0%、75.0%、0.0%、0.0%,more common in Ⅰ and Ⅱ grade,the ratio of clinical grading in MS-LCH was 13.2%、19.3%、30.3%、36.8%,more common in Ⅲ and Ⅳ grade,there was significant difference between two groups(P<0.001). the ratio of clinical grading in PLCH was 20.9%、18.6%、27.9%、32.6%,the ratio of clinical grading in children who lungs uninvolved was 3.0%、21.2%、33.3%、42.4%,both more common in Ⅲ and Ⅳ grade,there was no significant difference between two groups(P>0.05).3.There were 30 cases(90.9%) have the interstitial changes in lungs CT images, the main manifestations were decrease lucent of lungs(21/30,70.0%),ground-glass opacity(12/30,40.0%)and fine reticular opacities( 11/30, 36.7%), all of decrease lucent of lungs distribute diffuse,8 cases were more obvious in posterior lungs. 7 cases of ground-glass opacity distribute diffuse,2 cases were more obvious in posterior lungs. 7 cases of fine reticular opacities distribute diffuse.There were 16(48.5%)cases have lungs nodules and(or) cysts,including 10 cases nodules and 9 cases cysts. 3 cases of nodules and 1 cases of cysts distribute solitary, 8 cases of nodules and(or) cysts distribute interspersed,and 4 cases distribute diffuse. The solitary or interspersed lesions mostly in the subpleural(8/12,66.7%),interspersed lesions in both lungs, given priority to the lower lobe,middle and periphery. There were 2 cases(6.1%)have lungs fibrosis.4.The median age of disease onset of interstitial changes was 1.2 years old,and disease process was 2.5 months,the median age of disease onset of lungs nodules and(or) cysts was 0.9 years old,and disease process was 4 months,the disease onset was no significant difference between two groups(P>0.05),the disease process was significant difference between two groups(P=0.000).5. The rate of the lungs nodules and(or) cysts appeared among 4 clinical grading was 0.0% 、 57.1% 、 63.5% 、 35.7%, there was no significant difference between rate of the lungs nodules and(or) cysts appeared and clinical grading(P>0.05).The amount of lungs nodules and(or) cysts less than 10 were more common inⅡgrade(3/16,18.8%) and Ⅲ grade(3/16,18.8%),The amount of lungs nodules and(or) cysts more than 10 were more common in Ⅲ grade(4/16,25.0%) and Ⅳ grade(3/16,18.8%),there was no significant difference between two groups(P>0.05)。The interstitial changes distribute range Ⅰ grade from Ⅳ grade,more distribute in Ⅲ and Ⅳ grade,lungs nodules and(or) cysts distribute range Ⅱ grade from Ⅳ grade,1 case lungs fibrosis was Ⅲ grade and the other was Ⅳ grade, there were no significant difference between CT manifestations and clinical grading(P>0.05).Conclusions:1.In children,SS-LCH occurred frequently after 2 years old,many were skeleton involved alone.MS-LCH occurred frequently less than 2 years old, many organs/systems could involved, there were easily involoved organ were liver、skin and spleen.All of PLCH were MS-LCH, and always have skin involved.The main clinical grading of SS-LCH were Ⅰ、Ⅱ grade,the clinical grading of MS-LCH were more common in Ⅲ、Ⅳ grade.2. The interstitial changes were more common in lungs CT images of PLCH, the main manifestations were decrease lucent of lungs,ground-glass opacity and fine reticular opacities,have the feature of distribute diffuse and more obvious in posterior lungs.3.The lungs nodules and cysts were featured manifestations of PLCH,the lesions were solitary、interspersed or diffuse. The solitary or interspersed lesions mostly in the subpleural,interspersed lesions in both lungs,given priority to the lower lobe,middle and periphery.4.The disease process of interstitial changes was shorter than lungs nodules and(or) cysts.6. The rate of the lungs nodules and(or) cysts appeared and the amount of the lung nodules and(or) cysts didn’t increased with the rise of clinical grading.The CT manifestations seriousness didn’t exacerbate with the rise of clinical grading.