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[脊柱背侧朗格汉斯细胞组织细胞增多症。脊髓压迫症的一种罕见病因]

[Langerhans' cell histiocytosis of the dorsal spine. A rare etiology of spinal cord compression].

作者信息

Ghorbel I Ben, Boukhris I, Kchir M N, Abderrahmane K, Khanfir M, Lamloum M, Miled M, Houman M H

机构信息

Service de médecine interne, hôpital La Rabta, 1007 Tunis, Tunisia.

出版信息

Rev Med Interne. 2008 Jan;29(1):58-62. doi: 10.1016/j.revmed.2007.08.019. Epub 2007 Sep 25.

Abstract

INTRODUCTION

Langerhans cell histiocytosis of the thoracic spine is a rare condition in adults. It is a serious condition with an increased risk of spinal cord compression.

EXEGESIS

A 55-year-old man complained of lumbar and back pain. He developed a spastic paraparesis. Magnetic resonance imaging of the spine showed a spinal cord compression due to bone and epidural tumoral lesions of the eleventh and twelfth thoracic vertebrae, located in the posterior elements of the spine. A surgical biopsy showed an infiltrate of eosinophilic cells, positive for CD1a in immunohistochemistry studies. Final diagnosis was therefore langerhans cell histiocytosis of the thoracic spine complicated by spinal cord compression. The patient received glucocorticoid therapy combined with vinblastine followed by local radiotherapy and etoposide. However, the neurological deficit persisted and the patient finally died.

CONCLUSION

Langerhans cell histiocytosis is a rare cause of spinal cord compression.

摘要

引言

胸椎朗格汉斯细胞组织细胞增多症在成人中较为罕见。这是一种严重疾病,脊髓受压风险增加。

病例分析

一名55岁男性主诉腰背部疼痛。他出现了痉挛性截瘫。脊柱磁共振成像显示,由于位于脊柱后部结构的第十一和第十二胸椎的骨质及硬膜外肿瘤性病变导致脊髓受压。手术活检显示有嗜酸性细胞浸润,免疫组织化学研究中CD1a呈阳性。因此最终诊断为胸椎朗格汉斯细胞组织细胞增多症并发脊髓受压。患者接受了糖皮质激素联合长春花碱治疗,随后进行了局部放疗和依托泊苷治疗。然而,神经功能缺损持续存在,患者最终死亡。

结论

朗格汉斯细胞组织细胞增多症是脊髓受压的罕见原因。

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