研究动态
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高危真性红细胞增多症患者的脊髓髓外造血模拟硬膜外肿瘤:说明性病例。

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

发表日期:2024 Jul 08
作者: Ashlee N Hawkins, Jeffrey M Breton, Akrita Bhatnagar, Nitika Paudel, Jean-Marc Voyadzis
来源: MEDICINE & SCIENCE IN SPORTS & EXERCISE

摘要:

在此,作者介绍了一名 43 岁男性病例,有 T 细胞淋巴瘤病史,接受阿扎胞苷加环磷酰胺、阿霉素、长春新碱和泼尼松治疗以及自体造血细胞移植,并患有高危真性红细胞增多症。 PCV)表现为严重的腰痛,放射至双腿,并伴有下肢无力和脾肿大。T2加权磁共振成像显示涉及T1-10和S1-2的多节段硬膜外病变。由于椎管严重狭窄,患者接受了T5-7椎管减压手术,术后下肢疼痛立即缓解,小腿无力完全消失。活检结果显示髓外造血(EMH)类似于脊髓硬膜外肿瘤。 EMH 具有放射敏感性,并且对低剂量表现出快速反应,因此患者进一步接受姑息性放射治疗以消除残留肿瘤并缓解症状,并使用羟基脲和皮质类固醇进行细胞减灭术。EMH 与 PCV 或骨髓增殖性疾病相关。脊柱是一种罕见现象,没有标准的治疗方法。 https://thejns.org/doi/10.3171/CASE23659。
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.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.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.