Sugiyama Atsuhiko, Kobayashi Makoto, Daizo Ayaka, Suzuki Miyako, Kawashima Hirotoshi, Kagami Shin-Ichiro, Tanaka Hiroaki, Suzuki Yoshio, Matsunaga Takashi, Kuwabara Satoshi
Department of Neurology, Asahi General Hospital, Japan.
Department of Neurology, Graduate School of Medicine, Chiba University, Japan.
Intern Med. 2017;56(13):1715-1718. doi: 10.2169/internalmedicine.56.8051. Epub 2017 Jul 1.
An 87-year-old woman presented with a 3-month history of fever, edema of the lower legs, and gait disturbance. A laboratory examination revealed high serum levels of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA). Although microscopic polyangiitis was initially suspected and treated, the patient subsequently developed transient hemiparesis and disturbed consciousness. Brain magnetic resonance imaging/angiography revealed infarct-like lesions, pachymeningeal involvement, and diffuse cerebral vasoconstriction. A random skin biopsy confirmed the histological diagnosis of intravascular lymphoma. Diffuse cerebral vasoconstriction and a high serum MPO-ANCA level have rarely been reported in patients with intravascular lymphoma. Endothelial damage due to immune-mediated mechanisms, tumor derived factors, or the direct interaction of lymphoma cells with endothelial cells may commonly predispose patients to both cerebral vasoconstriction and the development of ANCAs.
一名87岁女性，有3个月的发热、小腿水肿和步态障碍病史。实验室检查显示血清髓过氧化物酶特异性抗中性粒细胞胞浆抗体（MPO-ANCA）水平升高。尽管最初怀疑并治疗了显微镜下多血管炎，但患者随后出现了短暂性偏瘫和意识障碍。脑磁共振成像/血管造影显示梗死样病变、硬脑膜受累和弥漫性脑血管收缩。随机皮肤活检确诊为血管内淋巴瘤。血管内淋巴瘤患者很少有弥漫性脑血管收缩和高血清MPO-ANCA水平的报道。免疫介导机制、肿瘤衍生因子或淋巴瘤细胞与内皮细胞的直接相互作用导致的内皮损伤，可能通常使患者易患脑血管收缩和ANCA的发生。
