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血管内淋巴瘤表现为长节段性脊髓炎:诊断挑战和病因线索。

Intravascular lymphoma presenting as a longitudinally-extensive myelitis: diagnostic challenges and etiologic clues.

机构信息

Dept. of Neurology, Mayo Clinic, Rochester, MN 55905, United States.

出版信息

J Neurol Sci. 2011 Apr 15;303(1-2):146-9. doi: 10.1016/j.jns.2010.12.016. Epub 2011 Jan 17.

DOI:10.1016/j.jns.2010.12.016
PMID:21247588
Abstract

Intravascular lymphoma is a rare disorder that commonly involves the central nervous system. Neurologic involvement may be the presenting and only manifestation. Identifying intravascular lymphoma as the cause of neurologic disease is diagnostically challenging. We report an elderly woman presenting with subacute onset paraparesis due to spinal cord involvement by an intravascular lymphoma. Progressive worsening was associated with extension of a longitudinally-extensive thoracic intramedullary spinal cord lesion. Extensive investigations failed to provide a diagnosis in life and repeated empiric therapeutic trials were unsuccessful. Diagnostic confirmation was postmortem. A longitudinally-extensive spinal cord lesion has a broad differential diagnosis. Intravascular lymphoma should be considered particularly in older individuals. The presence of coexisting hematologic abnormalities should prompt consideration of a bone marrow biopsy. Early diagnosis may direct therapy and lead to a more favorable prognosis.

摘要

血管内淋巴瘤是一种罕见的疾病,通常涉及中枢神经系统。神经受累可能是其首发和唯一表现。将血管内淋巴瘤确定为神经疾病的病因具有诊断挑战性。我们报告了一例老年女性,因血管内淋巴瘤累及脊髓而出现亚急性起病的截瘫。进行性恶化与胸髓内广泛的长节段脊髓病变的延伸有关。广泛的检查未能在生前明确诊断,反复经验性治疗试验均未成功。诊断是在死后得到确认的。长节段脊髓病变的鉴别诊断广泛。在老年人中,尤其应考虑血管内淋巴瘤。如果同时存在血液学异常,应提示行骨髓活检。早期诊断可能指导治疗并带来更好的预后。

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