# Intravascular large B-cell lymphoma

Source: https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/  
OnCo record `intravascular-large-b-cell-lymphoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Summary

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.

How common: no registry figure; 182 published cases in a decade (Cancer Management and Research 2020).

Treatment: R-CHOP with central nervous system-directed therapy (high-dose methotrexate and intrathecal chemotherapy) as in PRIMEUR-IVL; autologous transplant considered in first remission for fit patients; central nervous system disease treated as on the primary CNS lymphoma page.

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: IVLBCL; Intravascular lymphoma; Angiotropic large cell lymphoma; Malignant angioendotheliomatosis
- Tags: subtype-page; wave4; haematologic; rare
- Group: haematologic
- Burden: Very rare: a review of the world literature over 2008 to 2018 found 182 pathologically confirmed cases in 103 publications (Cancer Management and Research 2020). No registry figure exists.
- Subtypes: Intravascular large B-cell lymphoma, Western variant (neurological and cutaneous); Intravascular large B-cell lymphoma, Asian variant (haemophagocytic syndrome, marrow, cytopenias); Cutaneous variant (skin-limited, better prognosis)
- Biomarkers: Random skin biopsy or organ biopsy showing intravascular CD20-positive large cells; Lactate dehydrogenase and ferritin (haemophagocytic variant); Cerebrospinal fluid and MRI for central nervous system disease; MYD88 L265P and CD79B mutations (frequent)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/intravascular-large-b-cell-lymphoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/#what-it-is [3 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/#treating-it [1 setting, 1 decision with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/#science [1 target]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/#living-with-it [8 questions, 4 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/coming/ [3 medicines]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/data/ [15 connected records]

## Standard of care

- 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. ([Rituximab](https://onco.cc/drugs/rituximab/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Methotrexate](https://onco.cc/drugs/methotrexate/), [R-CHOP (lymphoma chemoimmunotherapy)](https://onco.cc/terms/r-chop/), [Primary CNS lymphoma](https://onco.cc/cancers/primary-cns-lymphoma/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Intravascular_lymphomas
- NCI PDQ: adult non-Hodgkin lymphoma treatment: https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq
- Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms: https://doi.org/10.1038/s41375-022-01620-2
- PRIMEUR-IVL (Shimada 2020, Lancet Oncology): R-CHOP with high-dose methotrexate and intrathecal chemotherapy in intravascular large B-cell lymphoma: https://doi.org/10.1016/s1470-2045(20)30059-0
- Cancer Management and Research 2020: prognosis of intravascular large B-cell lymphoma, 182 published cases: https://doi.org/10.2147/cmar.s267825

## Connected records

- cancers: [Diffuse large B-cell lymphoma](https://onco.cc/cancers/dlbcl/), [HIV-associated (AIDS-related) lymphomas](https://onco.cc/cancers/hiv-associated-lymphoma/), [Lymphomatoid granulomatosis](https://onco.cc/cancers/lymphomatoid-granulomatosis/), [Primary CNS lymphoma](https://onco.cc/cancers/primary-cns-lymphoma/)
- drugs: [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Methotrexate](https://onco.cc/drugs/methotrexate/), [Rituximab](https://onco.cc/drugs/rituximab/)
- terms: [MYD88 L265P and CXCR4 mutations](https://onco.cc/terms/myd88-l265p/), [R-CHOP (lymphoma chemoimmunotherapy)](https://onco.cc/terms/r-chop/)

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JSON: https://onco.cc/api/v1/entities/intravascular-large-b-cell-lymphoma.json