一例64岁男性急性脑病的奇怪病例

Joseph R. Malhis, S. O’Brien, Alex Belote, B. Brander
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摘要

64岁男性,急性脑病,经进一步评估,发现颈部和腮腺区有淋巴结病。随后的腰椎穿刺(LP)显示脑脊液中存在与白血病性脑膜炎有关的母细胞。淋巴结活检符合CD5阳性DLBCL。他开始接受Hyper-CVAD和鞘内甲氨蝶呤化疗,临床状况有所改善,但未能随访。弥漫性大b细胞淋巴瘤(DLBCL)是最常见的非霍奇金淋巴瘤(NHL)类型。诊断是基于症状和切除淋巴结活检与免疫分型。由于其侵袭性,如果怀疑淋巴瘤,进行淋巴结活检是非常重要的。其指标包括CD5阳性和结外受累。DLBCL中中枢神经系统受累的危险因素包括高国际预后指数(IPI)评分、骨髓受累和结外受累,如骨、肺和睾丸。该报告的目的是强调在脑病的背景下,当弥漫性淋巴结病变存在时,排除淋巴瘤的重要性;这种疾病的潜在攻击行为,包括累及中枢神经系统;并讨论该患者健康的整体社会决定因素,导致延误诊断和缺乏最佳治疗计划。
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Curious case of acute encephalopathy in a 64-yearold male
A 64-year-old male presents with acute encephalopathy and upon further evaluation, he was found to have lymphadenopathy in the neck and parotid region. Subsequent lumbar puncture (LP) revealed the presence of blast cells in the CSF concerning for leukemic meningitis. A lymph node biopsy was consistent with CD5 positive DLBCL. He was started on chemotherapy with Hyper-CVAD and intrathecal methotrexate, improving his clinical condition but he was lost to follow up. Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin lymphoma (NHL). Diagnosis is based on symptoms and excisional lymph node biopsy with immunotyping. It is extremely important to have a lymph node biopsy if suspicious for lymphoma because of its aggressive nature. Indicators for such include CD5 positivity and extranodal involvement. The risk factors for CNS involvement in DLBCL include high International Prognostic Index (IPI) score, bone marrow involvement, and extranodal involvement such as bone, lung and testis. The purpose of the report is to emphasize the importance of ruling out lymphoma when diffuse lymphadenopathy is present in the setting of encephalopathy; the potential of aggressive behavior of this disease including involvement of the CNS; and discussing the overall social determinants of health for this patient resulting in delayed diagnosis and lack of an optimal treatment plan to be carried out.
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