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在主动脉-双髂动脉旁路手术中发现的偶然致病性纤维蛋白相关弥漫性大B细胞淋巴瘤

Incidental Pathogenic Fibrin-Associated Diffuse Large B-cell Lymphoma Found During Aorto-Biiliac Bypass.

作者信息

Habib Peter M, Serena Thomas, Flynn Caitlin M, Hartkop Aaron, Wey Elizabeth, Lang David, Laveroni Eugene

机构信息

Department of Surgery, Beaumont Health, Farmington Hills, USA.

Department of Internal Medicine, Beaumont Health, Farmington Hills, USA.

出版信息

Cureus. 2022 Mar 31;14(3):e23681. doi: 10.7759/cureus.23681. eCollection 2022 Mar.

Abstract

Fibrin-associated diffuse large B-cell lymphoma (FA-DLBCL) is in and of itself a rare entity and is a subset of the Epstein-Barr virus (EBV)-associated lymphoma. Due to its indolent course, FA-DLBCL is generally an incidental finding on histopathological examinations. We present the first reported case of FA-DLBCL found within a native aortic thrombus during an aorto-biiliac bypass. This is a 77-year-old male who was taken to the operative theater for open aorto-biiliac bypass secondary to aortooclusive disease resulting in intermittent claudication and gangrene of the right lower extremity digits. Intraoperatively, suspicious inflammatory changes were noted around the aorta. Pathological evaluation of the thrombus within the aorta noted cells of B-cell lineage with BCL2 and MYC positivity in addition to CD30 and EBV positivity. Postoperatively, the patient's course was complicated by acute tubular necrosis, uremia, dialysis dependence, intubation, and cardiac arrhythmias including cardiac arrest. He was able to recover from these complications, however, he ultimately chose to self-enroll in hospice care. An extensive literature review of over 128 mentions of FA-DLBCL noted a complete paucity of reported cases of FA-DLBCL within a native aorta. The patient's clinical presentation and histopathology without mass-forming lesions lead to the diagnosis of FA-DLBCL. FA-DLBCL is an extremely rare EBV+ lymphoproliferative disorder associated with chronic inflammation (DLBCL-CI). FA-DLBCL is a rare condition without defined uniform treatment. This article serves to highlight the first reported case of FA-DLBCL found within an abdominal aortic thrombus in a native aorta. Given the paucity of literature on this condition, postoperative treatment and long-term outcomes should be the focus of this condition.

摘要

纤维蛋白相关弥漫性大B细胞淋巴瘤(FA-DLBCL)本身是一种罕见疾病,是与爱泼斯坦-巴尔病毒(EBV)相关淋巴瘤的一个亚型。由于其病程缓慢,FA-DLBCL通常是在组织病理学检查中偶然发现的。我们报告了首例在主-双髂动脉搭桥手术中发现于天然主动脉血栓内的FA-DLBCL病例。这是一名77岁男性,因主动脉闭塞性疾病导致间歇性跛行和右下肢趾端坏疽,被送往手术室进行开放性主-双髂动脉搭桥手术。术中,在主动脉周围发现可疑的炎症变化。对主动脉内血栓的病理评估发现,除了CD30和EBV阳性外,还有B细胞系细胞呈BCL2和MYC阳性。术后,患者出现急性肾小管坏死、尿毒症、依赖透析、插管以及包括心脏骤停在内的心律失常等并发症。不过,他从这些并发症中康复过来,但最终选择自行登记接受临终关怀。对超过128篇提及FA-DLBCL的文献进行广泛综述后发现,完全没有在天然主动脉内发生FA-DLBCL的报道病例。患者无形成肿块病变的临床表现和组织病理学表现导致了FA-DLBCL的诊断。FA-DLBCL是一种极其罕见的与慢性炎症相关的EBV阳性淋巴增殖性疾病(DLBCL-CI)。FA-DLBCL是一种罕见疾病,尚无明确统一的治疗方法。本文旨在突出首例在天然主动脉腹主动脉血栓内发现FA-DLBCL的报道病例。鉴于关于这种疾病的文献匮乏,术后治疗和长期预后应成为关注重点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c95d/9061222/4bcec856a83f/cureus-0014-00000023681-i01.jpg

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