Intravascular lymphoma: a role for single-agent rituximab

Area of Science:

• Hematology
• Oncology
• Neurology

Background:

• Intravascular lymphoma (IVL) is a rare, aggressive neoplasm.
• IVL often presents with neurological and cutaneous symptoms, complicating ante mortem diagnosis.

Observation:

• A 65-year-old man presented with altered mental status, fevers, and weight loss.
• Diagnosis was confirmed via skin biopsies showing intravascular, CD20-positive cells.
• Initial chemotherapy (cyclophosphamide, vincristine, doxorubicin, prednisone, rituximab) led to disease progression.

Findings:

• Switching to weekly single-agent rituximab resulted in a complete response and symptom resolution.
• The patient achieved a sustained remission for 3 years post-therapy.
• This is the first reported case of sustained remission with single-agent rituximab in neurologically affected IVL after anthracycline failure.

Implications:

• Single-agent rituximab demonstrates significant activity in treating intravascular lymphoma.
• Rituximab may be a viable treatment option for refractory or neurologically involved IVL.
• This case highlights the potential of targeted therapy in rare hematologic malignancies.