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模仿血管炎的血管内淋巴瘤。

Intravascular lymphoma mimicking vasculitis.

作者信息

Prayson Richard A

机构信息

Cleveland Clinic, Department of Anatomic Pathology, 9500 Euclid Avenue, Cleveland, OH 44195, USA.

出版信息

J Clin Neurosci. 2016 Dec;34:224-225. doi: 10.1016/j.jocn.2016.06.010. Epub 2016 Jul 26.

DOI:10.1016/j.jocn.2016.06.010
PMID:27469408
Abstract

Intravascular lymphoma is a rare malignancy which is characterized by a proliferation of atypical appearing B cells, generally confined to vascular lumina. A tissue biopsy demonstrating the pathology is required to make a diagnosis. The tumor is often disseminated at the time of diagnosis and prognosis is poor, even with aggressive chemotherapy. Neurologic presentations of this neoplasm can be quite varied. This report documents the presence of intravascular lymphoma diagnosed on a brain biopsy in a 60-year-old man. He initially presented 6months before brain biopsy with chest pain and hypotension, warranting coronary artery bypass graft surgery. Four months later, he presented with signs attributed to a stroke (diaphoresis, slumped over in a chair and left hand weakness). He subsequently developed a sudden onset wide-based gait, left leg numbness, word finding difficulties and worsening confusion. A MRI study showed multiple infarcts in the brain, including cerebellum. Invasive angiogram suggested vasculitis. He was started on a course of treatment for presumed central nervous system vasculitis. He continued to develop signs suggestive of ongoing infarct development and a biopsy from the right parietal was taken. The biopsy showed atypical intravascular CD20 positive staining B cells, consistent with intravascular lymphoma.

摘要

血管内淋巴瘤是一种罕见的恶性肿瘤,其特征是出现非典型的B细胞增殖,通常局限于血管腔。需要组织活检来证实病理情况以做出诊断。该肿瘤在诊断时往往已发生播散,即使采用积极的化疗,预后也很差。这种肿瘤的神经系统表现可能多种多样。本报告记录了一名60岁男性经脑活检确诊为血管内淋巴瘤的病例。他在脑活检前6个月最初因胸痛和低血压就诊,接受了冠状动脉搭桥手术。4个月后,他出现了类似中风的症状(出汗、瘫坐在椅子上以及左手无力)。随后,他突然出现宽基底步态、左腿麻木、找词困难以及意识障碍加重。磁共振成像(MRI)研究显示脑部包括小脑在内有多处梗死灶。侵入性血管造影提示血管炎。他开始接受针对假定的中枢神经系统血管炎的治疗疗程。他持续出现提示梗死灶不断发展的症状,于是对右侧顶叶进行了活检。活检显示非典型的血管内CD20阳性染色B细胞,符合血管内淋巴瘤的表现。

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Ann Neurol. 2025 Mar;97(3):435-448. doi: 10.1002/ana.27132. Epub 2024 Nov 28.
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Rare intravascular large B-cell lymphoma (IVLBCL) can cause atypical intracerebral haemorrhage and mislead diagnostics.罕见的血管内大 B 细胞淋巴瘤(IVLBCL)可引起非典型性颅内出血,从而导致误诊。
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