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中间型地中海贫血患者出现截瘫的髓外造血

Extramedullary haemopoiesis in thalassemia intermedia presenting as paraplegia.

作者信息

Tan Tze-Ching, Tsao Joanne, Cheung Fung-Ching

机构信息

Department of Neurosurgery, Queen Elizabeth Hospital, Kowloon, Hong Kong SAR, China.

出版信息

J Clin Neurosci. 2002 Nov;9(6):721-5. doi: 10.1054/jocn.2001.1038.

Abstract

Extramedullary haemopoiesis causing spinal cord compression is a rare manifestation of thalassemia. We describe a 17 year old male with thalassemia intermedia who presented with progressive paraplegia and sphincter disturbance. Magnetic resonance imaging revealed an epidural lesion extending from T5 to T8 compressing the spinal cord. The patient recovered completely after surgical decompression with postoperative radiation therapy. Histological examination of the lesion confirmed the diagnosis of extramedullary haemopoiesis. Clinical awareness of this phenomenon with early treatment is essential for optimizing the neurological outcome.

摘要

导致脊髓压迫的髓外造血是地中海贫血的一种罕见表现。我们描述了一名17岁中间型地中海贫血男性患者,其出现进行性截瘫和括约肌功能障碍。磁共振成像显示一个从T5延伸至T8的硬膜外病变压迫脊髓。该患者在手术减压并术后放疗后完全康复。病变的组织学检查确诊为髓外造血。对这种现象的临床认识以及早期治疗对于优化神经功能转归至关重要。

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