目的 探讨CT对郎格尔汉斯细胞组织细胞增生症(Langerhans cell histiocytosis,LCH)造成多脏器损害诊断的价值及限度。方法 分析13例经临床、实验室、病理检查确诊的LCH的CT表现。局限性LCH 4例，广泛性LCH 9例。全部病例均行颅脑、胸部、肝、脾CT平扫检查，4例行增强检查。结果 男性多于女性，颅穹隆骨破坏依次为颞骨8例、顶骨3例、枕骨2例、额骨1例。CT发现早期骨破坏敏感性高。肿块变化可反映病变由活跃增殖到静止消退的病理过程。下丘脑-垂体轴侵犯影像表现晚于临床。CT可显示LCH不同阶段的肺损害。结论 LCH各脏器损害的影像学表现缺乏特异性。好发部位的典型CT表现可提示诊断，同时对辅助LCH分型、确定治疗方案、估计预后有一定价值。%Objective To study the value and limitation of CT in the damage to multiple organs in children with Langerhans cell histiocytosis (LCH). Methods Analysis of CT manifestations was performed in 13 cases of LCH proved by clinical features, laboratory data, and pathologic changes. Localized LCH was found in 4 cases, and disseminated LCH in 9 cases. All cases were examined by plain scan in craniocerebral, chest, liver, and spleen, and four cases by contrast-enhanced CT scan. Results The male outnumbered female in this group. Cranial bones were involved in the sequence of temporal bone(8 cases), parietal bone (3 cases), occipital bone (2 cases), and frontal bone (1 case). CT findings were very sensitive in showing the early destructions of the bones. The characteristic manifestation on CT was multiple lytic lesions of the flat bones at any sites, without reactive sclerosis and soft tissue mass accompanied with the bony lesion. Imaging findings presented later than the clinical manifestations for LCH involving the hypothalamus/pituitary axis. Involvement of the lung at various stages by LCH could be demonstrated by CT scan. Conclusion Imaging manifestations had no characteristics in LCH involvement. The sites of predilection and related CT manifestations could be suggestive of the diagnosis. In addition, CT findings can be helpful in the classification and evaluation of the therapy and prognosis in LCH.
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