Intravascular large B-cell lymphoma: remission after rituximab-cyclophosphamide, doxorubicin, vincristine, and prednisolone chemotherapy.

Intravascular lymphoma is an uncommon, very aggressive extranodal non-Hodgkin lymphoma that most frequently involves the skin and central nervous system. Most cases are of B-cell origin; T-cell phenotype is extremely rare. Malignant cells proliferate within the lumens of capillaries, arterioles, venules, and small arteries; vascular occlusion is responsible for the clinical signs and symptoms. The prognosis of this high-grade B-cell lymphoma has improved since the introduction of the anti-CD20 monoclonal antibody, rituximab. We describe a case of B-cell intravascular lymphoma successfully treated with rituximab and cyclophosphamide, doxorubicin, vincristine, and prednisolone.