The Diving Bell and the Butterfly Revisited: A Fatal Case of Locked-in Syndrome in a Man With Epstein-Barr Virus-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified.

IF 3 Q2 Medicine
Clinical Medicine Insights-Blood Disorders Pub Date : 2018-03-26 eCollection Date: 2018-01-01 DOI:10.1177/1179545X18762799
Jacqueline N Poston, Russell Dorer, David M Aboulafia
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引用次数: 2

Abstract

Epstein-Barr virus (EBV)-positive diffuse large B-cell lymphoma (DLBCL) is a rare variant of DLBCL. The natural history of this subtype is poorly understood. Incomplete literature in the era of rituximab suggests that patients with EBV-positive DLBCL have similar outcomes to patients with EBV-negative DLBCL when treated with rituximab and anthracycline-based chemotherapy regimens; however, there are few prospective studies on this subtype and little is known about the risk of central nervous system (CNS) relapse with EBV-positive DLBCL. Herein, we describe the case of a 64-year-old man who presented with stage IIA EBV-positive DLBCL. His international age-adjusted International Prognostic Index (IPI) was 2. He achieved a complete response to 6 cycles of rituximab combined with chemotherapy consisting of dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, and doxorubicin. After 10 days of completion of chemotherapy, he had a fulminant neurologic decline manifested by diffuse weakness followed by a locked-in syndrome; he could only communicate by moving his eyes. He had been deemed at low risk for CNS relapse based on the application of the recently developed CNS-IPI score of 2 (1 point for age >60 years and 1 point for lactate dehydrogenase higher than normal) and consequently did not receive therapy for CNS prophylaxis. A limited postmortem autopsy revealed extensive lymphoma throughout the brain, particularly in the deep basal nuclei, midbrain, pons, centrum semiovale, and corpus callosum. This presentation of CNS relapse is rare and has not yet been described in EBV-positive DLBCL. We discuss some of the unique aspects of this case including the clinical manifestations of locked-in syndrome and its differential diagnosis and the uncertain benefits of CNS prophylaxis in this clinical context.

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潜水钟和蝴蝶重访:一名患有爱泼斯坦-巴尔病毒阳性弥漫性大b细胞淋巴瘤的男子闭锁综合征的致命病例,没有其他说明。
eb病毒(EBV)阳性弥漫大b细胞淋巴瘤(DLBCL)是一种罕见的DLBCL变体。这种亚型的自然历史尚不清楚。利妥昔单抗时代的不完整文献表明,接受利妥昔单抗和蒽环类化疗方案治疗时,ebv阳性DLBCL患者与ebv阴性DLBCL患者的结局相似;然而,关于该亚型的前瞻性研究很少,并且对ebv阳性DLBCL的中枢神经系统(CNS)复发的风险知之甚少。在这里,我们描述的情况下,64岁的男子谁提出了IIA期ebv阳性DLBCL。他的国际年龄调整国际预后指数(IPI)为2。利妥昔单抗联合由剂量调整依托泊苷、强的松、长春新碱、环磷酰胺和阿霉素组成的化疗6个周期后,患者完全缓解。化疗完成10天后,患者出现暴发性神经功能下降,表现为弥漫性虚弱,随后出现闭锁综合征;他只能通过转动眼睛来交流。根据最近开发的CNS- ipi评分为2分(年龄60岁1分,乳酸脱氢酶高于正常1分),他被认为是CNS复发的低风险,因此没有接受CNS预防治疗。有限的尸检显示广泛的淋巴瘤遍及大脑,特别是在深基底核、中脑、桥脑桥、半囊椎体和胼胝体。这种中枢神经系统复发的表现是罕见的,在ebv阳性的DLBCL中尚未被描述。我们讨论了这个病例的一些独特方面,包括闭锁综合征的临床表现及其鉴别诊断,以及在这种临床背景下中枢神经系统预防的不确定益处。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
3.70
自引率
0.00%
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审稿时长
8 weeks
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