Arboix A, Costa I, Besses C
Unidad de Patología Vascular Cerebral, Hospital del Sagrat Cor, Barcelona, España.
Rev Neurol. 2000;30(12):1188-90.
Intravascular lymphomatosis is a rarely seen clinicopathological condition.
To review the literature on cerebral ischemia and intravascular lymphomatosis.
Intravascular lymphomatosis is usually caused by an uncommon type of non-Hodgkin lymphoma, usually of B cells, characterized by the localization of predominantly neoplastic cells within the small calibre blood vessels (arterioles, venules and capillaries). Therefore, it tends to cause multifocal vascular occlusions, with symptoms generally limited to the central nervous system, in the form of recurrent, multifocal cerebral infarcts--one in every 5,000 consecutive cases is usually of this aetiology--or rapidly progressive encephalopathy. Cerebral or meningeal biopsy, or biopsy of peripheral nerve, muscle, adrenal or lymphoid tissue, prostate or lung is usually diagnostic. The differential diagnosis is with vasculitis, multi-infarct dementia, occult neoplasia or infections. In spite of starting suitable treatment with chemotherapy or radiotherapy, the average survival from onset of symptoms is usually four months.
Intravascular lymphomatosis should be taken into account in the differential diagnosis of repeated cerebral ischaemia of unusual aetiology.
血管内淋巴瘤病是一种罕见的临床病理状况。
回顾关于脑缺血与血管内淋巴瘤病的文献。
血管内淋巴瘤病通常由一种不常见类型的非霍奇金淋巴瘤引起,通常为B细胞淋巴瘤,其特征是主要肿瘤细胞定位于小口径血管(小动脉、小静脉和毛细血管)内。因此,它倾向于导致多灶性血管闭塞,症状通常局限于中枢神经系统,表现为复发性、多灶性脑梗死——每5000例连续病例中通常有1例为此病因——或快速进展性脑病。脑或脑膜活检,或外周神经、肌肉、肾上腺或淋巴组织、前列腺或肺的活检通常具有诊断意义。鉴别诊断需与血管炎、多发性梗死性痴呆、隐匿性肿瘤或感染相区分。尽管开始采用化疗或放疗进行适当治疗,但从症状出现开始计算的平均生存期通常为四个月。
在病因不明的反复脑缺血的鉴别诊断中应考虑血管内淋巴瘤病。