Intravascular lymphoma: a role for single-agent rituximab

Intravascular lymphoma is a rare aggressive systemic neoplasm with neurological and cutaneous presentation, which commonly eludes the diagnosis ante mortem . We document a case of intravascular lymphoma in a 65-year-old man who presented with altered mental status, fevers and weight loss. The diagnosis was made by random skin biopsies demonstrating an intravascular, CD20 positive cellular infiltrate. Initial treatment consisted of cyclophosphamide, vincristine, doxorubicin, prednisone and rituximab. Because of disease progression, the therapy was switched to weekly rituximab after the first cycle. A complete response was observed with resolution of symptoms and laboratory abnormalities. The patient remains in remission 3 years after completion of therapy. This is the first case report to describe a sustained remission following single-agent rituximab in the patient with neurologic involvement of intravascular lymphoma who failed antracycline-based therapy. Single-agent rituximab has activity in this disease and may be considered as a treatment option.