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中枢神经系统黏膜相关淋巴组织型原发性结外边缘区B细胞淋巴瘤(MZL CNS)表现为创伤性硬膜下血肿和蛛网膜下腔出血——病例报告

Primary extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue type in the central nervous system (MZL CNS) presented as traumatic subdural hematoma and subarachnoid bleeding - case report.

作者信息

Jesionek-Kupnicka Dorota, Smolewski Piotr, Kupnicki Piotr, Pluciennik Elzbieta, Zawlik Izabela, Papierz Wielislaw, Kordek Radzislaw

出版信息

Clin Neuropathol. 2013 Sep-Oct;32(5):384-92. doi: 10.5414/NP300579.

DOI:10.5414/NP300579
PMID:23557903
Abstract

The study describes a very rare case of primary extranodal marginal zone Bcell lymphoma of the central nervous system (MZL CNS) with an unusual clinical and radiological presentation mimicking subarachnoid bleeding and subdural hematoma (SDH) after head injury. The patient presented symptoms which had commenced 3 weeks earlier: a gradually-progressing headache associated with periodic right-sided cramp of the face muscles and numbness of the right upper limb. During urgent craniotomy for drainage of the presumed SDH, a tumor mass histopathologically and immunohistochemically matching marginal zone B-cell lymphoma was found. Molecular analysis confirmed monoclonal immunoglobulin heavy chain gene (IgH) rearrangement; the patient had previously suspected nodal lymphoma because of cervical lymphadenopathy, but histopathological, immunohistochemical and molecular examination excluded malignant lymphoma. The patient underwent successful radiotherapy, and achieved complete response. At present, no evidence of either systemic disease or lymph node enlargement has been found. The recognition of an indolent type of lymphoma in a rare anatomical localization is very important due to the proper management of the patient.

摘要

该研究描述了一例非常罕见的原发性中枢神经系统边缘区B细胞淋巴瘤(MZL CNS),其具有不寻常的临床和放射学表现,类似于头部受伤后的蛛网膜下腔出血和硬膜下血肿(SDH)。患者出现了3周前开始的症状:逐渐加重的头痛,伴有面部肌肉周期性右侧痉挛和右上肢麻木。在对疑似SDH进行紧急开颅引流时,发现了一个组织病理学和免疫组织化学上与边缘区B细胞淋巴瘤相符的肿瘤肿块。分子分析证实了单克隆免疫球蛋白重链基因(IgH)重排;患者此前因颈部淋巴结肿大曾怀疑患有淋巴结淋巴瘤,但组织病理学、免疫组织化学和分子检查排除了恶性淋巴瘤。患者接受了成功的放疗,并实现了完全缓解。目前,未发现全身疾病或淋巴结肿大的证据。由于对患者的正确管理,在罕见解剖部位识别惰性淋巴瘤类型非常重要。

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