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一名继发于系统性轻链淀粉样变性病的获得性X因子缺乏患者发生自发性脊髓硬膜外血肿。

Spontaneous spinal epidural hematoma in a patient with acquired Factor X deficiency secondary to systemic amyloid light-chain amyloidosis.

作者信息

Arishima Hidetaka, Tada Ayumi, Isozaki Makoto, Kitai Ryuhei, Kodera Toshiaki, Kikuta Ken-ichiro, Tai Katsunori, Iwasaki Hiromichi

出版信息

J Spinal Cord Med. 2015 Sep;38(5):641-4. doi: 10.1179/2045772314Y.0000000230. Epub 2014 Jun 29.

Abstract

BACKGROUND

Spontaneous spinal epidural hematoma (SSEH) is relatively rare. SSEH with anticoagulants including warfarin and rivaroxaban (Factor Xa inhibitor) have been reported; however, SSEH with Factor X deficiency has not been described yet.

METHODS

Case report.

FINDINGS

An 82-year-old woman with acquired Factor X deficiency complained of sudden onset of severe posterior neck pain. Magnetic resonance imaging demonstrated an epidural hematoma from C3 to T3 levels. Because she showed tetraparesis on the third hospital day, we performed surgery. Just before surgery, her prothrombin time-international normalized ratio was 2.49, which was immediately reversed by infusion of prothrombin complex concentrate. The patient safely underwent an emergency laminectomy from C3 to T2, in which the epidural hematoma was evacuated. Post-operatively, the patient recovered completely without rebleeding. Hematologists found acquired deficiency of Factor X in this patient with systemic amyloid light-chain amyloidosis.

CONCLUSION

To our knowledge, this is the first report of a case of SSEH with Factor X deficiency. A blood coagulation disorder should be considered in patients with SSEH.

摘要

背景

自发性脊髓硬膜外血肿(SSEH)相对罕见。已有使用包括华法林和利伐沙班(Xa因子抑制剂)在内的抗凝剂导致SSEH的报道;然而,X因子缺乏导致SSEH的情况尚未见描述。

方法

病例报告。

结果

一名患有获得性X因子缺乏的82岁女性突发严重颈后疼痛。磁共振成像显示C3至T3水平存在硬膜外血肿。由于她在住院第三天出现四肢轻瘫,我们进行了手术。就在手术前,她的凝血酶原时间-国际标准化比值为2.49,通过输注凝血酶原复合物浓缩剂立即得到纠正。患者安全地接受了从C3至T2的急诊椎板切除术,术中清除了硬膜外血肿。术后,患者完全康复,未再出血。血液科医生在该患有系统性轻链淀粉样变的患者中发现了获得性X因子缺乏。

结论

据我们所知,这是首例X因子缺乏导致SSEH的病例报告。SSEH患者应考虑存在凝血障碍。

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