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朗格汉斯细胞组织细胞增生症的骨骼受累。

Skeletal involvement in Langerhans cell histiocytosis.

机构信息

Department of Pediatric Radiology, Hôpital Jeanne de Flandre, Université de Lille 2, CHRU de Lille, Lille, France.

出版信息

Insights Imaging. 2013 Oct;4(5):569-79. doi: 10.1007/s13244-013-0271-7. Epub 2013 Aug 2.

Abstract

UNLABELLED

Langerhans cell histiocytosis (LCH) represents a disorder characterised by an abnormal accumulation of histiocytes in miscellaneous tissues. The bone is commonly affected, especially the flat bones, the spine and the long bones. Some lesions in children such as a "vertebra plana" or a solitary lytic lesion of the skull may be suggestive for LCH, whereas others can be confused with a malignant tumour or osteomyelitis. This pictorial essay presents the main usual and unusual skeletal manifestations observed in LCH.

TEACHING POINTS

• Osseous involvement in children with LCH is very similar to that seen in multiple myeloma. • A solitary lytic lesion of the cranial vault is a typical radiographic finding of LCH. • A vertebra plana appearance in the spine is another typical radiographic finding. • Extensive signal intensity changes within bone marrow on MRI are a helpful sign for the diagnosis. • In long bones, endosteal scalloping may be responsible for a "budding appearance".

摘要

目的

朗格汉斯细胞组织细胞增生症(LCH)是一种以组织细胞异常积聚为特征的疾病。骨骼是最常受累的部位,尤其是扁平骨、脊柱和长骨。儿童的一些病变,如“椎体扁平”或颅骨孤立性溶骨性病变,可能提示 LCH,而其他病变可能与恶性肿瘤或骨髓炎混淆。本文通过影像学表现介绍 LCH 常见和不常见的骨骼表现。

教学要点

  • LCH 患儿的骨骼受累与多发性骨髓瘤非常相似。

  • 颅盖骨的单一溶骨性病变是 LCH 的典型影像学表现。

  • 脊柱的“椎体扁平”表现是另一种典型的影像学表现。

  • MRI 上骨髓内广泛的信号强度改变有助于诊断。

  • 在长骨中,骨内膜扇贝样改变可能导致“芽状外观”。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/49c4/3781243/9905c2aee62e/13244_2013_271_Fig1_HTML.jpg

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