中枢神经系统血管内淋巴瘤病一例报告

目的通过对１例中枢神经系统血管内淋巴瘤病患者的临床、影像学和脑组织病理检查，探讨此病的诊断规律。方法对患者的临床症状及影像学改变进行系统观察，对脑手术标本进行组织学和免疫组织化学染色。结果患者，５０岁，男性。表现为亚急性脑病，病情迅速恶化，于病后４６天死亡。辅助检查发现血沉加快，脑脊液仅出现蛋白轻度升高。影像学检查显示大脑白质多灶性损害，随病程的延长，病灶迅速增大、增多。脑病理特点为广泛的血管内恶性Ｂ淋巴瘤细胞增殖，导致血管堵塞，白质内出现多发性陈旧及新鲜的缺血病灶。结论此病的诊断只能依靠脑病理学检查。临床鉴别诊断包括恶性胶质瘤、进行性多灶性白质脑病、多发性硬化和颅内血管炎。

Intravascular lymphomatosis of central nervous system

Objectives To study the diagnosis and differential diagnosis in a Chinese patient with intravascular lymphomatosis of central nervous system (CNSIVL). Methods CT and MRI were performed several times in the 50yearold, male patient with this disease. Cerebral biopsy was made and the specimens were stained histologically and immunohistologically. Results This patient experienced a sudden onset of ophthalmalgia on the right eye and headache on the right temporal area. During the following days he developed consciousness disturbance, disorientation, fever and abnormal behavior. He died 46 days after the onset of the symptoms. CT scanning revealed multifocal hypodensity lesions in the deep white matter. MRI demonstrated the lesions with long T1 and T2 signal, which were enhanced with contrast agents on T1 weighted images. The erythrocyte sedimentation rate was elevated. The cerebral spinal fluid findings were nonspecific. Morphologically, it was characterized by proliferation of neoplastic lymphocytes within the vessels producing vascular occlusion. Immunohistochemical study confirmed the Blymphocyte origin of nonHodgkin lymphoma. ConclusionsUpon our knowledge, this might be the first reported Chinese patient with CNSIVL diagnosed. The diagnosis of this disease depended on the pathological study. Progressive multifocal leucoencephalopathy, acute form of multiple sclerosis and cerebral vasculitis should be considered in the differential diagnosis of CNSIVL.