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真性红细胞增多症患者获得性血管性血友病综合征所致硬膜下出血

Subdural Hemorrhage Due to Acquired Von Willebrand Syndrome in a Patient With Polycythemia Vera.

作者信息

Shalaby Khalid, Correia Lima Jussie, Szulawski Robert

机构信息

Internal Medicine, University of Connecticut Health, Farmington, USA.

Neurology, Hartford Hospital, Hartford, USA.

出版信息

Cureus. 2021 Jul 25;13(7):e16625. doi: 10.7759/cureus.16625. eCollection 2021 Jul.

Abstract

Polycythemia vera (PV) is a chronic myeloproliferative neoplasm associated with thrombosis. A 48-year-old female with PV presented with right eye pain following a low-impact head trauma. She consumed aspirin for analgesia and took preparations of Chinese herbs. CT head revealed right-sided subdural hematoma. She had reduced Von Willebrand activity to 26%. Direct angiographic imaging showed an aneurysm arising from a right middle cerebral atery (MCA) branch. The patient was given 1-deamino-8-D-arginine vasopressin (DDAVP) prior to the craniotomy. Intra-operative examination revealed that the aneurysm-like structure was a small grape-like structure of the fibrinous part of the subdural membrane that had formed from the subdural hematoma. Acquired von Willebrand syndrome (AVWS) is an important risk factor for bleeding in PV. DDAVP may be useful to increase levels of Von Willebrand Factor (VWF) and decrease the risk of bleeding perioperatively. Exogenous substances such as ginseng should be investigated as possible contributors to bleeding tendency and discontinued.

摘要

真性红细胞增多症(PV)是一种与血栓形成相关的慢性骨髓增殖性肿瘤。一名48岁的PV女性患者在轻度头部外伤后出现右眼疼痛。她服用阿司匹林止痛并服用了中药制剂。头颅CT显示右侧硬膜下血肿。她的血管性血友病因子活性降至26%。直接血管造影成像显示一个动脉瘤起源于右侧大脑中动脉(MCA)分支。患者在开颅手术前接受了去氨加压素(DDAVP)治疗。术中检查发现,动脉瘤样结构是由硬膜下血肿形成的硬膜下膜纤维部分的一个小葡萄样结构。获得性血管性血友病综合征(AVWS)是PV患者出血的一个重要危险因素。DDAVP可能有助于提高血管性血友病因子(VWF)水平并降低围手术期出血风险。应调查人参等外源性物质是否可能导致出血倾向并停用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/23fd/8384727/22d05a3a5f56/cureus-0013-00000016625-i01.jpg

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