A Arboix1, I Costa, C Besses. 1. Unidad de Patología Vascular Cerebral, Hospital del Sagrat Cor, Barcelona, España.
Abstract
INTRODUCTION: Intravascular lymphomatosis is a rarely seen clinicopathological condition. OBJECTIVE: To review the literature on cerebral ischemia and intravascular lymphomatosis. DEVELOPMENT: 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. CONCLUSION: Intravascular lymphomatosis should be taken into account in the differential diagnosis of repeated cerebral ischaemia of unusual aetiology.
INTRODUCTION:Intravascular lymphomatosis is a rarely seen clinicopathological condition. OBJECTIVE: To review the literature on cerebral ischemia and intravascular lymphomatosis. DEVELOPMENT: 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. CONCLUSION:Intravascular lymphomatosis should be taken into account in the differential diagnosis of repeated cerebral ischaemia of unusual aetiology.