König C W, Pfannenberg C, Trübenbach J, Remy C, Böhmer G M, Ruck P, Claussen C D
Department of Diagnostic Radiology, University of Tübingen, Hoppe-Seyler-Strasse 3, 72076 Tübingen, Germany.
Eur Radiol. 2001;11(12):2516-20. doi: 10.1007/s003300100835. Epub 2001 Mar 1.
Langerhans' cell histiocytosis (LCH) is a disorder of histiocytic proliferation that primarily affects infants. Imaging findings of a rare case of lung and liver involvement in an adult are presented. High-resolution computed tomography (HRCT) of the lungs showed confluent thin-walled cystic air spaces compatible with advanced LCH. Liver CT and MRI revealed unspecific signs of fatty infiltration. Irregular widening of peripheral bile ducts was displayed in breath-hold MR cholangiography. This pattern is considered characteristic for sclerosing cholangitis and should support the diagnosis of LCH in case of concomitant cystic pulmonary disease, even in adult patients.
朗格汉斯细胞组织细胞增多症（LCH）是一种主要影响婴儿的组织细胞增殖性疾病。本文报道了一例罕见的成人肺和肝受累的影像学表现。肺部高分辨率计算机断层扫描（HRCT）显示融合的薄壁囊状气腔，符合晚期LCH表现。肝脏CT和MRI显示脂肪浸润的非特异性征象。屏气磁共振胰胆管造影显示外周胆管不规则增宽。这种表现被认为是硬化性胆管炎的特征性表现，在合并囊性肺部疾病时，即使是成年患者，也应支持LCH的诊断。
