伴有进行性中枢神经系统出血和多发夹层的血管内淋巴瘤。

IF 0.9 Q4 CLINICAL NEUROLOGY
Case Reports in Neurological Medicine Pub Date : 2020-01-10 eCollection Date: 2020-01-01 DOI:10.1155/2020/6134830
Ricky Chen, Gurjeet Singh, J Scott McNally, Cheryl A Palmer, Adam de Havenon
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引用次数: 5

摘要

简介:血管内淋巴瘤(IVL)是一种罕见且经常致命的疾病,其特征是血管内淋巴瘤细胞在腔内增殖。由于异质的临床表现和缺乏敏感的诊断方案,IVL的诊断通常是在尸检时做出的。然而,通过早期诊断和适当的化疗,预后大大改善,完全缓解是可能的。为了扩大IVL的可能表现,我们报告了一位活检证实的血管内大b细胞淋巴瘤的非典型表现的患者,他患有颅内和颅外动脉夹层以及进行性颅内出血。病例报告。一位47岁的女性表现为单侧感觉异常。她出现进行性多灶性梗死和出血,颅内和颅外动脉夹层,导致昏迷。脑活检显示IVL。她接受了积极的化疗,神经系统恢复良好,病情完全缓解。结论:已知IVL对小动脉和毛细血管的病理作用,但未引起大血管的解剖。诊断应考虑在病例不明原因的动脉夹层和进展性中风。通过适当的实验室筛查和组织活检确认的早期诊断可大大改善预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Intravascular Lymphoma with Progressive CNS Hemorrhage and Multiple Dissections.

Intravascular Lymphoma with Progressive CNS Hemorrhage and Multiple Dissections.

Intravascular Lymphoma with Progressive CNS Hemorrhage and Multiple Dissections.

Intravascular Lymphoma with Progressive CNS Hemorrhage and Multiple Dissections.

Introduction: Intravascular lymphoma (IVL) is an uncommon and often fatal disease characterized by intraluminal proliferation of lymphomatous cells within blood vessels. Because of a heterogeneous clinical presentation and lack of sensitive diagnostic protocols, diagnosis of IVL is most often made at autopsy. However, with early diagnosis and appropriate chemotherapy, the prognosis is greatly improved and complete remission is possible. In order to broaden the possible presentations of IVL, we present a patient with an atypical manifestation of biopsy-proven intravascular large B-cell lymphoma who suffered dissections of both intracranial and extracranial arteries in addition to progressive intracranial hemorrhages. Case Report. A 47-year-old woman presented with unilateral paresthesias. She developed progressive multifocal infarcts and hemorrhage with dissections of both intracranial and extracranial arteries, resulting in coma. Brain biopsy revealed IVL. She received aggressive chemotherapy and remains in complete remission with good neurologic recovery.

Conclusion: IVL is known to exert its pathology on small arteries and capillaries, but is not known to cause dissections of large vessels. The diagnosis should be considered in cases with unexplained arterial dissections and progressive strokes. Early diagnosis with appropriate laboratory screening and tissue confirmation by biopsy can lead to greatly improved outcomes.

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