Berthet Elodie, Guillonnet Antoine, Houillier Caroline, Ursu Renata, Soussain Carole, Touat Mehdi, Gueguen Antoine, de Renzis Benoît, Bigaut Kevin, Ahle Guido, Durozard Pierre, Grosset-Janin Deborah, Oberic Lucie, Bonnet Antoine, Grandjean Anne-Pascale, Moluçon-Chabrot Cécile, Hoang-Xuan Khê, Chabriat Hugues, Guey Stéphanie
Neurology Departement, Lariboisière Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
Neuroradiology Departement, Lariboisière Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France.
Ann Neurol. 2025 Mar;97(3):435-448. doi: 10.1002/ana.27132. Epub 2024 Nov 28.
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.
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.
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.
The results of this study highlight "red flags" that could help accelerate the diagnosis of intravascular lymphoma involving the CNS. ANN NEUROL 2025;97:435-448.
血管内淋巴瘤是B细胞淋巴瘤的一种罕见亚型，其特征为克隆性增殖局限于小血管腔内。超过50%的患者出现中枢神经系统（CNS）受累，但由于缺乏特征性表现，诊断往往延迟。我们旨在通过一项深入的队列研究，确定早期诊断伴有CNS受累的血管内淋巴瘤的关键表型特征。
我们建立了一个多中心回顾性队列，纳入了与法国眼脑淋巴瘤专家网络（LOC网络）合作招募的17例患者，并对病历数据进行回顾性分析。
在该队列中，17例患者中有15例（88%）出现局灶性神经发作，通常呈波动和/或反复性，68%的发作起病突然，提示血管源性。17例患者中有15例（88%）出现快速认知衰退，17例中有8例（47%）出现精神症状，17例中有14例（82%）出现“B征”。脑部MRI显示，16例患者中有14例（87%）出现多形性液体衰减反转恢复序列（FLAIR）高信号，16例患者中有13例（81%）出现扩散加权成像（DWI）阳性病变，这些病变随时间累积，具有缺血性病变的异常特征（进行性生长，1个月内持续DWI高信号，周围有更广泛的FLAIR高信号）。超过90%的病例观察到早发性炎症综合征和乳酸脱氢酶（LDH）水平升高。轻度且不一致的脑膜炎与几乎持续的脑脊液蛋白升高形成对比。7例患者中有4例（57%）白细胞介素10/6比值超过0.7,6例患者中有3例（50%）经皮肤活检获得病理诊断。
本研究结果突出了一些“危险信号”，有助于加速对累及CNS的血管内淋巴瘤的诊断。《神经病学纪事》2025年；97:435 - 448。
