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1例以角膜缘综合征为表现并经骨髓活检确诊的血管内恶性淋巴瘤病

[A case of intravascular malignant lymphomatosis presenting with epicornus syndrome and diagnosed by bone marrow biopsy].

作者信息

Tsugawa Jun, Tsuboi Yoshio, Inoue Hirosato, Ishizuka Kenji, Takeshita Shigemori, Yamada Tatsuo

机构信息

Department of Pathology, Faculty of Medicine, Fukuoka University, 7-45-1 Nanakuma, Johnan-ku, Fukuoka 814-0180, Japan.

出版信息

Brain Nerve. 2010 Mar;62(3):269-72.

Abstract

A-46-year-old woman admitted to our hospital because of numbness of bilateral lower limbs and urinary incontinence. The initial neurological examination showed sensory impairment below S1 level with urinary incontinence, indicating epicornus syndrome. Spinal MR imaging demonstrated unremarkable on conventional and enhanced images with Gd-DTPA. Cerebrospinal fluid examination revealed slightly elevated protein level without pleocytosis. Thereafter, subacute ascending myelopathy including flaccid paraparesis and urinary retention, developed. Because the patient had low grade fever, fatigue, weight loss and elevated serum soluble IL-2 receptor and LDH titers, we investigated her for lymphoma. Although lymphadenopathy or mass lesions were not found on whole-body CT scan, bone marrow biopsy showed the presence of inravascular large B-cell lymphoma (IVL). Thus the patient's progressive myelopathy was probably caused by IVL invasion. Ten days after the initiation of chemotherapy, her neurological symptoms transiently improved; however, her paraparesis and urinary incontinence gradually worsened thereafter, despite of treatment. IVL often presents with neurological manifestations, including myelopathy. There have been a few reports of IVL presenting with lower lumbar spinal cord and conus medullaris. It should be noted that IVL can cause unidentified progressive ascending myelopathy without positive MRI findings.

摘要

一名46岁女性因双下肢麻木和尿失禁入住我院。初始神经学检查显示S1水平以下感觉障碍伴尿失禁,提示马尾综合征。脊柱磁共振成像在常规及钆喷酸葡胺增强图像上未见明显异常。脑脊液检查显示蛋白水平轻度升高,无细胞增多。此后,患者出现亚急性上升性脊髓病,包括弛缓性截瘫和尿潴留。由于患者有低热、疲劳、体重减轻,血清可溶性白细胞介素-2受体和乳酸脱氢酶水平升高,我们对其进行了淋巴瘤检查。尽管全身CT扫描未发现淋巴结病或肿块病变,但骨髓活检显示存在血管内大B细胞淋巴瘤(IVL)。因此,患者进行性脊髓病可能是由IVL浸润引起的。化疗开始10天后,她的神经症状短暂改善;然而,尽管接受了治疗,她的截瘫和尿失禁此后仍逐渐加重。IVL常表现为神经症状,包括脊髓病。有少数关于IVL表现为下腰椎脊髓和脊髓圆锥病变的报道。应注意的是,IVL可导致无MRI阳性发现的不明原因进行性上升性脊髓病。

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