儿童朗格汉斯细胞组织细胞增生症的临床与肺部影像学表现分析

发布时间：2018-05-03 05:20

本文选题：朗格汉斯细胞组织细胞增生症 + 儿童　；参考：《重庆医科大学》2016年硕士论文

【摘要】：目的:探讨儿童朗格汉斯细胞组织细胞增生症(Langerhans Cell Histiocytosis,LCH)的临床以及肺部影像表现特点,分析其临床与肺部影像表现的关系,提高对该病诊断能力。方法:回顾性分析重庆医科大学附属儿童医院2008年1月~2015年12月确诊为LCH患儿140例,男79例,女61例,发病年龄0~15.4岁。根据临床表现和疾病浸润范围,将LCH分为单系统LCH(Single-System Langerhans Cell Histiocytosis,SS-LCH)和多系统LCH(Multi-System Langerhans Cell Histiocytosis,MS-LCH)两组,根据肺部有无受累,将MS-LCH分为肺朗格汉斯细胞组织细胞增生症(Pulmonary Langerhans Cell Histiocytosis,PLCH)和未累及肺部MS-LCH两组;临床根据年龄、受浸润器官数目以及危险器官有无功能受累三大因素将该病分为4级,分别分析各组的临床及肺部CT表现特点,比较临床分级与肺部CT表现的相关性。结果:1.140例患儿中SS-LCH64例,48例(75%)2岁发病,58例(90.6%)表现为骨骼系统的单独受累;MS-LCH76例,46例(60.5%)≤2岁发病,各器官均可受累,以肝脏(59/76,77.6%)、皮肤(57/76,75.0%)、脾脏(49/76,64.5%)受累常见。MS-LCH中PLCH33例,皮肤受累29例(87.9%),未累及肺部43例,皮肤受累28例(65.1%),两组患儿皮肤受累差异具有统计学意义(P0.05)。2.SS-LCH临床分级Ⅰ~Ⅳ级的比例分别为25.0%、75.0%、0.0%、0.0%,以Ⅰ、Ⅱ级为主,MS-LCHⅠ~Ⅳ级的比例分别为13.2%、19.3%、30.3%、36.8%,以Ⅲ、Ⅳ级多见,两组差异有统计学意义(P0.001)。PLCH临床分级Ⅰ~Ⅳ级的比例分别为20.9%、18.6%、27.9%、32.6%,未累及肺部的MS-LCH临床分级Ⅰ~Ⅳ级的比例分别为3.0%、21.2%、33.3%、42.4%,两组均以Ⅲ、Ⅳ级多见,差异无统计学意义(P0.05)。3.33例PLCH中,肺部CT表现(1)肺间质病变30例(90.9%),以肺透光度减低(21/30,70.0%)、“磨玻璃”影(12/30,40.0%)、细网格状影(11/30,36.7%)表现为主,21例肺透光度减低、7例“磨玻璃”影、7例细网格状影呈双肺广泛分布,8例肺透光度减低、2例“磨玻璃”影以双肺后部明显。(2)肺部结节影和(或)气囊影16例(48.5%),其中结节影10例,气囊影9例;3例结节影、1例气囊影呈孤立分布,8例结节影和(或)气囊影呈多个散在分布,4例呈弥漫分布;孤立或多个散在结节影、气囊影以胸膜下分布(8/12,66.7%)为主,弥漫性结节影和(或)气囊影以双肺上叶及外、中带明显。(3)肺纤维化2例(6.1%)。4.肺部CT表现为肺间质病变的患儿中位发病年龄为1.2岁,病程中位数为2.5月,结节影和(或)气囊影的患儿中位发病年龄为0.9岁,病程中位数为4月,两组发病年龄差异无统计学意义(P0.05),两组病程长短差异有统计学意义(P=0.000)。5.33例PLCH中临床分级Ⅰ~Ⅳ级的结节影和(或)气囊影检出率分别为0.0%、57.1%、63.6%、35.7%,各临床分级检出率差异无统计学意义(P0.05)。结节影和(或)气囊影数量≤10个的患儿临床分级以Ⅱ级(3/16,18.8%)、Ⅲ级(3/16,18.8%)多见,病变数量10个的患儿临床分级以Ⅲ级(4/16,25.0%)、Ⅳ级(3/16,18.8%)多见,两组比较差异不显著(P0.05)。肺间质病变在临床分级Ⅰ~Ⅳ级中均有分布,Ⅲ、Ⅳ级分布较多,结节影和(或)气囊影分布为Ⅱ~Ⅳ级,肺纤维化分布Ⅲ、Ⅳ级各1例,肺部CT表现的严重程度与临床分级的差异无统计学意义(P0.05)。结论:1.SS-LCH常2岁发病,以骨骼单独受累多见,临床分级以Ⅰ、Ⅱ级为主;MS-LCH多≤2岁发病,各器官均可受累,以肝脏、皮肤、脾脏受累多见,临床分级以Ⅲ、Ⅳ多见;PLCH均为MS-LCH,常伴有皮肤受累;2.PLCH肺部CT表现以肺间质病变多见,常见肺透光度减低、“磨玻璃”影、细网格状影,具有双肺广泛分布,且肺后部病变表现明显的特点;3.肺部结节影、气囊影是PLCH的特征性表现,病变可孤立、散在或弥漫分布;孤立或散在分布结节影、气囊影以胸膜下多见,弥漫性结节影和(或)气囊影以双肺上叶及外、中带为著;4.PLCH肺部CT间质病变表现早于结节影和(或)气囊影;5.肺部CT结节影和(或)气囊影的检出率及数量不随临床分级的增高而增多;PLCH肺部CT表现不随着临床分级的增高而加重。
[Abstract]:Objective: To explore the clinical and pulmonary imaging features of Langerhans Cell Histiocytosis (LCH) in children's Langerhans cell, and to analyze the relationship between the clinical and pulmonary imaging features, and to improve the diagnostic ability of the disease. Methods: a retrospective analysis of the Affiliated Children's Hospital of Medical University Of Chongqing was diagnosed as L in December, January 2008. 140 children with CH, 79 men and 61 women, 0~15.4 years of age. According to the clinical manifestations and the range of disease, the LCH was divided into two groups of single system LCH (Single-System Langerhans Cell Histiocytosis, SS-LCH) and multisystem LCH (Multi-System Langerhans). The Pulmonary Langerhans Cell Histiocytosis (PLCH) and the lung MS-LCH two groups were not involved. The clinical and pulmonary CT manifestations of each group were analyzed according to age, the number of infiltrating organs and the three major factors of the dangerous organ involvement. The clinical and pulmonary CT features were analyzed, and the clinical classification and lung CT performance were compared. Results: 1.140 cases of SS-LCH64, 48 cases (75%) 2 years of age, 58 cases (90.6%) of individual involvement in the skeletal system; MS-LCH76 cases, 46 cases (60.5%) less than 2 years of age, all organs can be involved, the liver (59/76,77.6%), skin (57/76,75.0%), spleen (49/76,64.5%) involvement in common.MS-LCH in PLCH33 cases, skin involvement 29 cases (87.9%), There were no pulmonary involvement in 43 cases and 28 cases of skin involvement (65.1%). The difference in skin involvement in the two groups was statistically significant (P0.05) the proportion of.2.SS-LCH clinical grade I to IV was 25%, 75%, 0%, and 0%, and the proportion of grade I to grade II was 13.2%, 19.3%, 30.3%, 36.8%, respectively, with grade III and IV, and there were statistical differences between the two groups. The proportion of P0.001.PLCH clinical grade I to grade I ~ IV were 20.9%, 18.6%, 27.9%, 32.6%, and the proportion of MS-LCH clinical grade I to IV of the lung was 3%, 21.2%, 33.3%, 42.4%, and two groups were all more common in class III and IV (P0.05).3.33 case PLCH, pulmonary CT manifestation (1) pulmonary interstitial lesions 30 cases (90.9%), lung (1), lung Light transmittance decreased (21/30,70.0%), "glass" shadow (12/30,40.0%), fine mesh shadow (11/30,36.7%), 21 cases of lung light transmittance decreased, 7 cases of "grinding glass" shadow, 7 cases of fine gridding in double lung widely distributed, 8 cases of lung permeability reduction, 2 cases of "glass" shadow in the posterior part of the lungs. (2) 16 cases of pulmonary nodules and (or) airbag shadow (4) 8.5%) among them, there were 10 cases of nodules, 9 cases with air bag shadow, 3 cases of nodule shadow, 1 cases with solitary airbag shadow, 8 cases of nodular shadow and (or) airbag shadow scattered in distribution, 4 cases diffuse distribution, solitary or multiple scattered in the nodule shadow, air bag shadow distribution (8/12,66.7%) as the main, diffuse nodule shadow and (or) air bag shadow in the upper and outer, middle band of double lung (3) 2 cases of pulmonary fibrosis (6.1%).4. pulmonary CT manifestations of pulmonary interstitial lesions in children with a median age of 1.2 years, a median of 2.5 months in the course of disease, 0.9 years of age in children with nodular shadow and (or) air bag shadow, the median of the course of disease in April, and the difference between the two groups of age differences (P0.05), and the difference between the two groups in the duration of the disease. The clinical classification of grade I ~ IV in.5.33 PLCH was 0%, 57.1%, 63.6%, 35.7%, respectively, and there was no significant difference between the clinical classification detection rates (P0.05). The number of patients with nodular shadow and / or air bag shadow was less than 10 (3/16,18.8%), grade III (3/16,18.8%), and the number of lesions was 1. The clinical grades of 0 children were grade III (4/16,25.0%) and grade IV (3/16,18.8%), and the difference between the two groups was not significant (P0.05). The pulmonary interstitial lesions were distributed in grade I to IV, the distribution of grade III and IV was more, the distribution of nodules and (or) airbags was grade II to IV, the distribution of pulmonary fibrosis in 1 cases, and the severity of CT in the lung. There was no statistically significant difference from clinical classification (P0.05). Conclusion: 1.SS-LCH often occurs at 2 years of age and is mostly involved in bone involvement. The clinical classification is mainly in grade I and II; MS-LCH is more than 2 years old. All organs can be involved with liver, skin and spleen, and the clinical classification is III and IV; PLCH is MS-LCH, often accompanied by skin involvement; 2.PLCH lung. CT manifestations were common in the pulmonary interstitial lesions, common pulmonary transmittance reduction, "grind glass" shadow, fine gridding shadow, with a wide distribution of two lungs, and obvious characteristics of the posterior lung lesions; 3. pulmonary nodules, and airbag shadows were characteristic manifestations of PLCH, isolated and diffuse distribution; isolated or scattered in the distribution of nodules, airbag shadow to chest The presence of diffuse nodules and (or) airbag shadows in the upper and outer areas of the lungs was more common. The 4.PLCH pulmonary CT interstitial lesions were earlier than the nodules and (or) airbag shadows; the detection rates and numbers of CT nodules and (or) airbags in the lungs were not increased with the increase of clinical classification; the CT manifestations of the PLCH lung did not increase with the increase of clinical classification.

【学位授予单位】：重庆医科大学
【学位级别】：硕士
【学位授予年份】：2016
【分类号】：R725

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