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髓外造血所致进行性脊髓病:病例报告及文献复习

Progressive myelopathy due to extramedullary hematopoiesis: case report and review of the literature.

作者信息

Stahl S M, Ellinger G, Baringer J R

出版信息

Ann Neurol. 1979 May;5(5):485-9. doi: 10.1002/ana.410050515.

Abstract

Extramedullary hematopoiesis (EMH) in the spinal epidural space is a rare but treatable cause of progressive paraparesis in patients with a variety of hematological and systemic disorders. We report a case of extensive thoracic epidural EMH causing progressive spastic paraparesis in a patient with agnogenic myeloid metaplasia. The literature describes 11 additional cases of myelopathy due to epidural EMH. All patients had EMH in the midthoracic region with elevated cerebrospinal fluid protein and abnormal spine roentgenograms or myelograms. Treatment with decompressive laminectomy, radiation therapy, or both, resulted in marked or complete recovery in 7 of 10 patients. Patients who develop paraparesis and who have a disorder associated with EMH should be evaluated rapidly for this treatable condition.

摘要

脊髓硬膜外间隙的髓外造血(EMH)是各种血液系统和全身性疾病患者进行性双下肢轻瘫的一种罕见但可治疗的病因。我们报告一例广泛的胸段硬膜外EMH导致一名原发性骨髓化生患者出现进行性痉挛性双下肢轻瘫的病例。文献中还描述了另外11例因硬膜外EMH导致脊髓病的病例。所有患者胸段中部均有EMH,脑脊液蛋白升高,脊柱X线片或脊髓造影异常。10例患者中有7例接受减压性椎板切除术、放射治疗或两者联合治疗后,症状显著改善或完全恢复。出现双下肢轻瘫且患有与EMH相关疾病的患者应迅速接受评估,以确定是否存在这种可治疗的疾病。

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