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肺朗格汉斯细胞组织细胞增生症:儿童和成人 CT 比较研究。

Pulmonary Langerhans cell histiocytosis: a comparative study of computed tomography in children and adults.

机构信息

Department of Diagnostic Imaging, The Ottawa Hospital, Ottawa, ON, Canada.

出版信息

J Thorac Imaging. 2012 Jan;27(1):65-70. doi: 10.1097/RTI.0b013e3181f49eb6.

DOI:10.1097/RTI.0b013e3181f49eb6
PMID:21042223
Abstract

PURPOSE

Langerhans cell histiocytosis is a rare idiopathic disorder, characterized by the infiltration of 1 or more organs by large mononuclear cells. It can develop at any age. Pulmonary Langerhans cell histiocytosis (PLCH) is found more frequently and is better described in adults than in children. The PLCH findings on computed tomography (CT) scans include nodular opacities, thin-walled cysts (often bizarre in shape), and marked parenchymal fibrosis. The purpose of this study was to compare the chest CT findings between adults and children with PLCH.

MATERIALS AND METHODS

We retrospectively analyzed the chest CT findings in a series of 7 children and 12 adults. Two independent observers reviewed the CT studies of all cases, and then correlated their observations. Statistical analysis was done with the Fisher exact test.

RESULTS

The mean ages were 8 years (3 mo to 16 y) for the pediatric group and 39 years (21 to 59 y) for the adults. Extrapulmonary disease was present in 3 (43%) children and 1 (8.3%) adult. All the adults reported smoking, and only 1 pediatric patient (16 y old) had a history of smoking. The CT findings were characterized by the presence of cysts, fibrosis, and nodules, with the findings being similar in size and appearance for both populations. However, the distribution was different; the subpleural parenchyma in the costophrenic recess was spared in the entire adult group, but not spared in any of the pediatric patients.

CONCLUSIONS

The CT findings of PLCH were similar in the adult and the pediatric populations, with the exception that costophrenic recess subpleural parenchyma was spared in the adult group but demonstrated pathology in the pediatric group.

摘要

目的

朗格汉斯细胞组织细胞增生症是一种罕见的特发性疾病,其特征是 1 个或多个器官被大单核细胞浸润。它可以在任何年龄发病。肺朗格汉斯细胞组织细胞增生症(PLCH)在成人中比在儿童中更为常见且描述更为详细。PLCH 的 CT 扫描表现包括结节状阴影、薄壁囊肿(形状常很奇特)和明显的实质纤维化。本研究的目的是比较成人和儿童 PLCH 的胸部 CT 表现。

材料与方法

我们回顾性分析了一组 7 例儿童和 12 例成人的胸部 CT 表现。两名独立观察者对所有病例的 CT 研究进行了复查,并对他们的观察结果进行了相关性分析。采用 Fisher 确切检验进行统计学分析。

结果

儿童组的平均年龄为 8 岁(3 个月至 16 岁),成人组的平均年龄为 39 岁(21 岁至 59 岁)。3 例(43%)儿童和 1 例(8.3%)成人存在肺外疾病。所有成人均报告有吸烟史,仅有 1 例儿童(16 岁)有吸烟史。CT 表现为囊肿、纤维化和结节,两组的表现大小和外观相似。然而,分布不同;整个成人组的肋膈角胸膜下实质未受累,但无任何儿童患者未受累。

结论

除了成人组肋膈角胸膜下实质未受累而儿童组有病变外,PLCH 的 CT 表现在成人和儿童群体中相似。

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