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一名难治性铁粒幼细胞贫血患者发生脊髓硬膜外髓外造血并伴有脊髓受压。病例报告。

Spinal epidural extramedullary hematopoiesis with cord compression in a patient with refractory sideroblastic anemia. Case report.

作者信息

Heffez D S, Sawaya R, Udvarhelyi G B, Mann R

出版信息

J Neurosurg. 1982 Sep;57(3):399-406. doi: 10.3171/jns.1982.57.3.0399.

Abstract

Spinal cord compression by epidural extramedullary hematopoiesis (EMH) is a rare phenomenon. A case of acute compressive myelopathy is reported in a 72-year-old man with EMH secondary to sideroblastic anemia. Technetium colloid scanning was used to document extensive ectopic marrow formation. The patient improved following surgery and radiotherapy. A review of the literature revealed 23 other cases of symptomatic spinal epidural EMH. The underlying hematological disorder varied but was always of long duration. Eighty-eight percent of the patients were males. Symptoms lasted longer than 1 week in 90% of cases, and 91% demonstrated incomplete neurological deficits. Plain x-ray films were rarely helpful in establishing the diagnosis. Technetium sulfur colloid bone marrow scanning has been used successfully to detect EMH and has led to preoperative diagnosis in one case. Decompressive laminectomy with or without postoperative irradiation is the suggested therapy, although there is evidence that radiotherapy alone may be adequate in some cases. Good recovery is the rule despite long-standing neurological deficits.

摘要

硬膜外髓外造血(EMH)导致的脊髓压迫是一种罕见现象。本文报道了一例72岁男性因铁粒幼细胞性贫血继发EMH而出现急性压迫性脊髓病的病例。使用锝胶体扫描记录广泛的异位骨髓形成。患者在手术和放疗后病情改善。文献回顾显示另有23例有症状的脊髓硬膜外EMH病例。潜在的血液系统疾病各不相同,但病程总是很长。88%的患者为男性。90%的病例症状持续超过1周,91%表现为不完全神经功能缺损。普通X线片在确立诊断方面很少有帮助。锝硫胶体骨髓扫描已成功用于检测EMH,并在一例中实现了术前诊断。建议采用减压性椎板切除术,术后可联合或不联合放疗,尽管有证据表明在某些情况下单纯放疗可能就足够了。尽管存在长期神经功能缺损,但通常恢复良好。

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