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高危真性红细胞增多症患者中模仿硬膜外肿瘤的脊髓髓外造血:病例说明

Spinal extramedullary hematopoiesis mimicking an epidural tumor in a patient with high-risk polycythemia vera: illustrative case.

作者信息

Hawkins Ashlee N, Breton Jeffrey M, Bhatnagar Akrita, Paudel Nitika, Voyadzis Jean-Marc

机构信息

Georgetown University School of Medicine, Washington, DC.

Departments of Neurosurgery, MedStar Georgetown University Hospital, Washington, DC.

出版信息

J Neurosurg Case Lessons. 2024 Jul 8;8(2). doi: 10.3171/CASE23659.

Abstract

BACKGROUND

Here the authors present the case of a 43-year-old male with a history of T-cell lymphoma, which was treated with azacitidine plus cyclophosphamide, doxorubicin, vincristine, and prednisone and autologous hematopoietic cell transplant, and high-risk polycythemia vera (PCV) presenting with severe lower-back pain radiating to the bilateral legs with associated lower-extremity weakness and splenomegaly.

OBSERVATIONS

T2-weighted magnetic resonance imaging revealed multilevel epidural lesions involving T1-10 and S1-2. Because of severe spinal canal stenosis, the patient underwent surgical decompression of T5-7, with immediate postoperative alleviation of the lower-extremity pain and complete resolution of the lower-leg weakness. Biopsy results revealed extramedullary hematopoiesis (EMH) mimicking a spinal epidural tumor. EMH is radiosensitive and displays a rapid response to low dosages, so the patient was further treated with palliative radiation therapy for residual tumors and symptom alleviation, as well as hydroxyurea and corticosteroids as indicated for cytoreduction.

LESSONS

EMH associated with PCV or myeloproliferative conditions occurring within the spine is a rare phenomenon without a standard treatment approach. https://thejns.org/doi/10.3171/CASE23659.

摘要

背景

本文作者报告了一例43岁男性病例,该患者有T细胞淋巴瘤病史,曾接受阿扎胞苷联合环磷酰胺、多柔比星、长春新碱和泼尼松治疗以及自体造血细胞移植,现患有高危真性红细胞增多症(PCV),表现为严重的下背部疼痛并放射至双侧腿部,伴有下肢无力和脾肿大。

观察结果

T2加权磁共振成像显示T1 - 10和S1 - 2节段存在多发硬膜外病变。由于严重的椎管狭窄,患者接受了T5 - 7节段的手术减压,术后下肢疼痛立即缓解,小腿无力完全消失。活检结果显示为模仿脊髓硬膜外肿瘤的髓外造血(EMH)。EMH对放疗敏感,对低剂量放疗反应迅速,因此患者接受了姑息性放射治疗以缓解残留肿瘤和症状,同时根据需要使用羟基脲和皮质类固醇进行细胞减灭治疗。

经验教训

与PCV或骨髓增殖性疾病相关的脊柱内EMH是一种罕见现象,目前尚无标准治疗方法。https://thejns.org/doi/10.3171/CASE23659

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