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[中枢神经系统淋巴瘤样肉芽肿病]

[Central nervous system lymphomatoid granulomatosis].

作者信息

Ogata Akihiko

机构信息

Department of Neurology, Hokkaido University Graduate School of Medicine.

出版信息

Rinsho Shinkeigaku. 2002 Nov;42(11):1127-30.

Abstract

Lymphomatoid granulomatosis (LYG) is a rare multiple organs disease with characteristic angiocentric lymphoproliferative features, most frequently involving the lung, skin, and rarely central nervous system. Most of the systemic LYG were shown as Epstein-Barr virus-associated, T-cell-rich, B-cell proliferative disorder. Based on radiological and pathological characteristics, we analyzed LYG-suspected four cases. The MRI features of LYG showed multiple punctate or linear enhancements that reside along the perivascular space. Pathologically, some cases showed borderline malignancy different from malignant lymphoma. LYG will be determined more clearly by the gene rearrangement studies in addition to radiological and immunohistochemical verifications, which will serve as indications for detection of monoclonality, further prognosis and therapy.

摘要

淋巴瘤样肉芽肿病(LYG)是一种罕见的多器官疾病,具有特征性的血管中心性淋巴增殖性特征,最常累及肺部、皮肤,很少累及中枢神经系统。大多数系统性LYG表现为与Epstein-Barr病毒相关的、富含T细胞的B细胞增殖性疾病。基于影像学和病理学特征,我们分析了4例疑似LYG的病例。LYG的MRI特征表现为沿血管周围间隙分布的多个点状或线状强化。病理上,一些病例表现出与恶性淋巴瘤不同的交界性恶性。除了影像学和免疫组化验证外,基因重排研究将更清楚地确定LYG,这将为单克隆性检测、进一步的预后和治疗提供指征。

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