Angioendotheliomatosis proliferans systemisata

A rare extranodal B-cell non-Hodgkin lymphoma, characterized by the presence of lymphoma cells exclusively in the lumina of small vessels, particularly capillaries. This is an extremely aggressive lymphoma which responds poorly to chemotherapy. (WHO, 2001) -- 2003

Disease Alternative Name

intravascular large b-cell lymphoma
angiotropic large cell lymphoma
intravascular b-cell lymphoma
malignant angioendotheliomatosis
angiotropic lymphoma
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