Szots Mónika, Bors László, Kálmán Endre, Szapáry László, Illés Zsolt
Pécsi Tudományegyetem, Altalános Orvostudományi Kar, Neurológiai Klinika, Pécs.
Orv Hetil. 2007 Apr 22;148(16):749-52. doi: 10.1556/OH.2007.27974.
Intravascular lymphomatosis is a rare systemic disease characterized by proliferation of malignant B or rarely T lymphocytes. Skin and the brain are predominantly affected. We describe a patient presenting with focal neurological signs and progressive dementia. Cerebral neuroimaging findings were nonspecific. Postmortem examination revealed intravascular proliferation of atypical mononuclear cells in the lumens of small vessels in all organs. The authors conclude that diagnosis requires a high index of suspicion and pathological examination of the affected organs, but is rarely made ante mortem.
血管内淋巴瘤病是一种罕见的全身性疾病，其特征为恶性B淋巴细胞或罕见的T淋巴细胞增殖。皮肤和脑是主要受累部位。我们描述了一名出现局灶性神经体征和进行性痴呆的患者。脑部神经影像学检查结果无特异性。尸检发现所有器官的小血管腔内有非典型单核细胞的血管内增殖。作者得出结论，诊断需要高度的怀疑指数和对受累器官进行病理检查，但生前很少能做出诊断。
