Unveiling the Clinical and Imaging Signatures of Intravascular Lymphoma of the Central Nervous System: A Multicentric Cohort Study.

IF 8.1 1区 医学 Q1 CLINICAL NEUROLOGY
Elodie Berthet, Antoine Guillonnet, Caroline Houillier, Renata Ursu, Carole Soussain, Mehdi Touat, Antoine Gueguen, Benoît de Renzis, Kevin Bigaut, Guido Ahle, Pierre Durozard, Deborah Grosset-Janin, Lucie Oberic, Antoine Bonnet, Anne-Pascale Grandjean, Cécile Moluçon-Chabrot, Khê Hoang-Xuan, Hugues Chabriat, Stéphanie Guey
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引用次数: 0

Abstract

Objective: Intravascular lymphoma is a rare subtype of B-cell lymphoma characterized by a clonal proliferation restricted to the lumen of small vessels. Over 50% of patients exhibit central nervous system (CNS) involvement, but diagnosis is often delayed due to the lack of distinctive features. We aimed to identify key phenotypic features for early diagnosis of intravascular lymphoma with CNS involvement through an in-depth cohort study.

Methods: We built up a multicenter retrospective cohort of 17 patients recruited in collaboration with the French Expert Network for Oculo-Cerebral Lymphomas (LOC network), and retrospectively analyzed data from medical records.

Results: In this cohort, 15 of 17 (88%) patients developed focal neurological episodes, often fluctuating and/or recurrent, with a sudden onset in 68% of episodes, suggesting a vascular origin. Rapid cognitive deterioration occurred in 15 of 17 (88%) patients, psychiatric manifestations in 8 of 17 (47%), and "B signs" in 14 of 17 (82%). Brain MRI showed polymorphic FLAIR hyperintensities in 14 of 16 (87%) patients, and DWI-positive lesions in 13 of 16 (81%) of patients, which accumulated over time and had unusual characteristics for ischemic lesions (progressive growth, persistent DWI-hyperintensity over 1 month, surrounded by a wider FLAIR hyperintensity). Early-onset inflammatory syndrome, and elevated lactate dehydrogenase (LDH) levels were observed in over 90% of cases. Mild and inconsistent meningitis contrasted with a nearly-constant hyperproteinorachia. An increased interleukin 10/6 ratio over 0,7 was found in 4 of 7 (57%) patients, and skin biopsy led to a pathological diagnosis in 3 of 6 (50%) patients.

Interpretation: The results of this study highlight "red flags" that could help accelerate the diagnosis of intravascular lymphoma involving the CNS. ANN NEUROL 2024.

揭示中枢神经系统血管内淋巴瘤的临床和影像学特征:多中心队列研究
目的:血管内淋巴瘤是一种罕见的 B 细胞淋巴瘤亚型:血管内淋巴瘤是B细胞淋巴瘤的一种罕见亚型,其特点是局限于小血管腔内的克隆增殖。50%以上的患者表现为中枢神经系统(CNS)受累,但由于缺乏明显特征,诊断往往被延误。我们旨在通过一项深入的队列研究,确定早期诊断中枢神经系统受累的血管内淋巴瘤的关键表型特征:我们与法国眼脑淋巴瘤专家网络(LOC网络)合作,建立了一个多中心回顾性队列,共招募了17名患者,并对病历数据进行了回顾性分析:在这批患者中,17 名患者中有 15 名(88%)出现了局灶性神经症状,这些症状通常具有波动性和/或复发性,其中 68% 的症状是突然发作的,这表明病因与血管有关。17例患者中有15例(88%)出现认知能力迅速退化,8例(47%)出现精神症状,14例(82%)出现 "B征"。脑磁共振成像显示,16 名患者中有 14 名(87%)出现多形性 FLAIR 高密度,16 名患者中有 13 名(81%)出现 DWI 阳性病变,这些病变随着时间的推移不断累积,并具有缺血性病变的异常特征(进行性生长,DWI 高密度持续 1 个月以上,周围有更广泛的 FLAIR 高密度)。90%以上的病例早期出现炎症综合征,乳酸脱氢酶(LDH)水平升高。轻微而不稳定的脑膜炎与几乎恒定的高蛋白血症形成鲜明对比。7例患者中有4例(57%)发现白细胞介素10/6比值升高超过0.7,6例患者中有3例(50%)通过皮肤活检获得病理诊断:这项研究的结果凸显了一些 "红旗",有助于加快中枢神经系统血管内淋巴瘤的诊断。ann neurol 2024.
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来源期刊
Annals of Neurology
Annals of Neurology 医学-临床神经学
CiteScore
18.00
自引率
1.80%
发文量
270
审稿时长
3-8 weeks
期刊介绍: Annals of Neurology publishes original articles with potential for high impact in understanding the pathogenesis, clinical and laboratory features, diagnosis, treatment, outcomes and science underlying diseases of the human nervous system. Articles should ideally be of broad interest to the academic neurological community rather than solely to subspecialists in a particular field. Studies involving experimental model system, including those in cell and organ cultures and animals, of direct translational relevance to the understanding of neurological disease are also encouraged.
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