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Intravascular large B-cell lymphoma (IVLBL) is an uncommon form of non-Hodgkin lymphoma that is also known as malignant angioendotheliosis, intravascular lymphomatosis, and angiotropic large-cell lymphoma. The disease is characterized by a bizarre population of neoplastic cells, which are found systemically within vascular lumina. Although originally thought to be a neoplastic process of the endothelial cells, it has since been demonstrated, by molecular techniques and immunohistochemistry, that the neoplastic cells are of lymphoid origin.

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Neoplastic angioendotheliosis (NAE) is a rare neoplastic disease, and its pre-mortem diagnosis is extremely difficult. A 49-year-old male developed vertigo, hearing and visual disturbance, transverse myelopathy below Th 5 and hypercalcemia. These symptoms were markedly improved by VEPA chemotherapy.

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Neoplastic angioendotheliosis has rarely been described as a respiratory disease. A patient is described with pulmonary vascular involvement induced by neoplastic angioendotheliosis in pulmonary vessels.

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Cerebral angiotropic large cell lymphoma (neoplastic angioendotheliosis) is a rare disease with a particular propensity to affect the central nervous system by vascular occlusion. Because the disease is rare and there are no specific diagnostic procedures apart from cerebral biopsy, it is difficult to diagnose in life. Accordingly, chemotherapy or radiotherapy has only rarely been attempted and their effectiveness is uncertain.

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Gallium scintigraphy in neoplastic angioendotheliosis of the lung.

Clin Nucl Med

January 1991

Department of Radiology, Saitama Medical Center, Saitama Medical School, Japan.

A case of neoplastic angioendotheliosis of the lung is reported. A 56-year-old man presented with a 2-month history of fever, increased serum lactic dehydrogenase, and arterial hypoxemia. Chest radiography revealed diffuse reticulonodular opacity, whereas CT scanning showed diffuse small nodular areas of increased attenuation with indistinct borders.

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