Inoue Daisuke, Hamamura Takeshi, Uehara Taira, Miyoshi Katsue, Katsuragi Takefumi, Takeshita Iwao, Hamada Tetsuo
Department of Neurosurgery, Kyushu Rosai Hospital, 1-3-1 Kuzuharatakamatsu, Kokuraminami-ku, Kitakyushu-shi, Fukuoka 800-0296, Japan.
No Shinkei Geka. 2009 Apr;37(4):369-74.
A 60-year-old female with intravascular lymphoma (IVL) presented with the complaint of urinary dysfunction and gait disturbance. T2 weighted MR imaging of the thoracic spinal cord showed a hyperintense lesion, and brain MR imaging indicated hyperintense lesions in the deep white matter. Multiple sclerosis was assumed, so steroid pulse therapy was administered. However, her level of consciousness decreased and her paraplegia progressed. Laboratory data showed that anemia and thrombocytopenia had worsened with high serum LDH and soluble IL-2 receptor levels. Biopsy of bone marrow indicated hypercellularity associated with hemophagocytic histiocytes, although no atypical lymphocytes were detected. Brain MR imaging indicated a new subcortical lesion in the left parietal lobe. One and a half months after admission, an open brain biopsy of the left parietal cortex was performed. Histopathological diagnosis was IVL, large B cell type. Immediately, she underwent CHOP therapy containing rituximab (R-CHOP therapy). After chemotherapy, spinal and brain MR images showed no new abnormal lesions. Clinically, it is difficult to make a diagnosis of IVL in life as it has no characteristic symptoms or radiological findings. Therefore, if a patient is suspected of having IVL, a biopsy of different organs, including brain, is necessary for making an early diagnosis and initiating chemotherapy.
一名60岁患有血管内淋巴瘤(IVL)的女性因排尿功能障碍和步态不稳前来就诊。胸椎脊髓的T2加权磁共振成像显示有一个高信号病变,脑部磁共振成像显示深部白质有高信号病变。考虑为多发性硬化症,于是给予了类固醇脉冲疗法。然而,她的意识水平下降,截瘫加重。实验室检查数据显示,贫血和血小板减少加重,血清乳酸脱氢酶(LDH)和可溶性白细胞介素-2受体水平升高。骨髓活检显示细胞增多,并伴有噬血细胞组织细胞,尽管未检测到非典型淋巴细胞。脑部磁共振成像显示左顶叶出现一个新的皮质下病变。入院一个半月后,对左顶叶皮质进行了开放性脑活检。组织病理学诊断为IVL,大B细胞型。随即,她接受了含利妥昔单抗的CHOP方案治疗(R-CHOP方案)。化疗后,脊髓和脑部磁共振图像未显示新的异常病变。临床上,由于IVL没有特征性症状或影像学表现,很难在生前做出诊断。因此,如果怀疑患者患有IVL,对包括脑在内的不同器官进行活检对于早期诊断和启动化疗是必要的。