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原发性中枢神经系统淋巴瘤伴发脱髓鞘及克雅氏星形胶质细胞

Primary Central Nervous System Lymphoma with Associated Demyelination and Creutzfeldt Astrocytes.

作者信息

Achi Hanadi El, Lally Jason E, Riascos Roy F, Zhu Jay-Jiguang, Tandon Nitin, Esquenazi Yoshua, Bhattacharjee Meenakshi B, Ballester Leomar Y

机构信息

Departments of Pathology, Texas Medical Center, Houston TX, USA.

Radiology, Texas Medical Center, Houston TX, USA.

出版信息

Ann Clin Lab Sci. 2018 Jul;48(4):534-537.

Abstract

A previously healthy 54-year-old woman presented with weight loss, progressive weakness that was more pronounced on the left side, and intermittent occipital headaches. Imaging studies showed multiple enhancing lesions along the white matter, compatible with a demyelinating process. The patient's previous history included relapsing-remitting symptoms of weakness over the past 3 years. A stereotactic brain biopsy showed histological features of demyelination with an associated population of neoplastic lymphoid cells. These unusual findings raise the question of whether demyelinating disease preceded the development of primary CNS lymphoma (PCNSL), or whether PCNSL induced demyelination. Although rare, cases of "sentinel lesions" heralding the diagnosis of PCNSL have been reported. This case emphasizes the importance of having a high index of suspicion of PCNSL in the setting of suspected demyelinating lesion in an adult patient.

摘要

一名54岁既往健康的女性出现体重减轻、进行性肌无力(左侧更为明显)以及间歇性枕部头痛。影像学检查显示沿白质有多个强化病灶,符合脱髓鞘过程。患者既往史包括过去3年出现的复发-缓解型肌无力症状。立体定向脑活检显示有脱髓鞘的组织学特征以及相关的肿瘤性淋巴细胞群。这些不寻常的发现引发了一个问题,即脱髓鞘疾病是先于原发性中枢神经系统淋巴瘤(PCNSL)发生,还是PCNSL诱发了脱髓鞘。尽管罕见,但已有报道称存在预示PCNSL诊断的“前哨病变”病例。该病例强调了在成年患者疑似脱髓鞘病变的情况下,对PCNSL保持高度怀疑指数的重要性。

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