ABSTRACT
Intravascular large B-cell lymphoma (IV-LBL) belongs to the group of diffuse large B-cell lymphomas, other, and in most of the cases, IVL is a systemic disease from the very beginning. It is a rare high-grade malignant type of extranodal large cell lymphoma, characterized by the presence of tumor cells in the lumina of small vessels, particularly capillaries and venules especially of the skin and the nervous system. The tumor cells express B-cell markers in the vast majority of cases. Rare tumors have a T-cell phenotype (see Comments). Other synonyms include systemic angioendotheliomatosis (1), intravascular lymphomatosis, and Tappeiner-Pfleger syndrome. This rare disease was initially regarded as a neoplastic proliferation of endothelial cells. Neoplastic angioendotheliomatosis has to be differentiated from reactive angioendotheliomatosis, which demonstrates a wide clinicopathologic spectrum in conjunction with a variety of underlying systemic inflammatory or neoplastic diseases (2,3).
CLINICAL FEATURES
