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首页> 外文期刊>Allergy, Asthma & Clinical Immunology >Multiple hepatic aneurysms and dry gangrene of fingertips in eosinophilic granulomatosis with polyangiitis: a case report
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Multiple hepatic aneurysms and dry gangrene of fingertips in eosinophilic granulomatosis with polyangiitis: a case report

机译:嗜嗜酸性粒细胞粒细胞瘤纤维炎的多发性肝动脉瘤和干坏疽性案例报告

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摘要

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic necrotizing vasculitis mainly affecting small-sized arteries. Involvement of medium-sized vessels is very rare in EGPA. Here we present the case of a patient with EGPA who showed multiple hepatic aneurysms and distal gangrene. A known EGPA patient visited to the emergency room (ER) with abrupt squeezing abdominal pain. She had suffered from gangrene in the fingertips of both hands for 1 year because of arterial thrombosis associated with hypereosinophilia. However, her absolute eosinophil count in the ER was 1120 cells/μL. An abdomen-pelvis CT demonstrated subcapsular hematoma in the right hepatic lobe. A celiac angiogram demonstrated multiple sized aneurysms in both hepatic lobes and some aneurysms in S7 and S8 were huge, more than 1?cm in size. The shape of the small aneurysms resembled a string of beads, as in polyarteritis nodosa. Given the clinical situation, emergency embolization was performed. Before this patient visited to the ER, she had been treated with a high dose of systemic corticosteroid, azathioprine, and cyclophosphamide. After addition of mepolizumab, the eosinophil count remained stable state with a near zero percentage of total white blood cell count. Aneurysm and gangrene resulting from the involvement of medium-sized vessels can occur in EGPA. Destruction of vessels might occur even if eosinophil count is below 1500 cells/μL. If involvement of medium-sized arteries is suspected, thorough investigation to identify the involved organs and prompt management are needed to prevent fatal complications.
机译:嗜酸性粒细胞芽孢杆菌(EGPA)是一种系统性坏死性,主要影响小型动脉。中尺寸血管的参与在EGPA中非常罕见。在这里,我们呈现出患有EGPA的患者,该患者显示多个肝动脉瘤和远端坏疽。一个已知的EGPA患者访问急诊室(ER),突然挤压腹痛。由于与低渗粒细胞有关的动脉血栓形成,她在双手的指尖中遭受了Gangrene。然而,ER中的绝对嗜酸性粒细胞计数是1120个细胞/μl。腹部骨盆CT在右肝叶中显示出亚面血肿。乳糜血管血管仪在肝裂片中展示多个尺寸的动脉瘤,S7和S8中的一些动脉瘤均为巨大,大小超过1Ωcm。小动脉瘤的形状类似于珠子的串,如多种细菌炎。鉴于临床情况,进行紧急栓塞。在该患者访问ER之前,她已经用高剂量的全身皮质类固醇,氮杂唑和环磷酰胺治疗。在加入Mepolizumab之后,嗜酸性粒细胞计数保持稳定状态,近零均为白细胞计数。由中型血管累及引起的动脉瘤和坏疽可以在EGPA中发生。即使嗜酸性粒细胞计数低于1500个细胞/μL,也可能发生血管的破坏。如果怀疑中尺寸动脉的参与,则需要彻底调查识别所涉及的器官和及时管理以防止致命并发症。

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