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4 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Intravascular large B-cell lymphoma is a rare form of large B-cell lymphoma in which the cancer cells grow inside small blood vessels rather than forming lumps, so it causes fevers, confusion, skin patches or breathlessness and is often found late or only after death. Rituximab-based chemotherapy with drugs that reach the brain has turned a nearly always fatal disease into one often controlled.
WHO-HAEM5 lists intravascular large B-cell lymphoma among the large B-cell lymphomas as an entity defined by selective growth of tumour cells within the lumina of small vessels, with a Western variant dominated by neurological and skin involvement and an Asian variant with haemophagocytic syndrome, marrow involvement and cytopenias (Alaggio 2022). In 182 published cases, one- and three-year overall survival were 42.3 and 11.5 percent with a median of 340 days; rituximab-containing regimens lengthened overall survival (450 against 180 days) and progression-free survival, while blood-brain-barrier-penetrating drugs gave no extra benefit for disease already in the central nervous system (Cancer Management and Research 2020). The Japanese phase 2 PRIMEUR-IVL trial gave R-CHOP with high-dose methotrexate and intrathecal chemotherapy to prevent central nervous system relapse in untreated patients without central nervous system disease (Shimada 2020, linked here).
How it differs from its parent: no mass and usually no lymphadenopathy, so diagnosis rests on random skin biopsy, marrow or organ biopsy; frequent central nervous system relapse, so prophylaxis is built into first-line treatment; and a high rate of haemophagocytic syndrome in the Asian variant.
| Setting | Approach | Guideline |
|---|---|---|
| All cases | R-CHOP with high-dose methotrexate and intrathecal chemotherapy (PRIMEUR-IVL); autologous transplant considered in first remission; CNS disease as on the primary CNS lymphoma page. | not mapped |