# Lymphomatoid granulomatosis

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

## TL;DR

Lymphomatoid granulomatosis is a rare Epstein-Barr virus-driven disease of B cells that invades and destroys blood vessels, almost always in the lungs and often the brain and skin, in people whose immune control of the virus is weak. Low-grade disease can be treated with interferon and high-grade disease as a large B-cell lymphoma with rituximab-based chemotherapy.

## Summary

WHO-HAEM5 lists lymphomatoid granulomatosis among the EBV-positive B-cell lymphoproliferative disorders, graded 1 to 3 by the number of large EBV-positive B cells, with grade 3 overlapping large B-cell lymphoma (Alaggio 2022; Katzenstein 2010). It affects mainly middle-aged adults, men almost twice as often as women, with multiple bilateral lung nodules and extrapulmonary involvement of skin and nervous system in more than a third; mortality has been high and treatment was not well established (Katzenstein 2010). In the National Cancer Institute series of 55 patients, all had lung involvement, 38 percent central nervous system involvement, none nodal or marrow disease, and all had past EBV exposure with a low median viral load; the lesions were angiocentric and rich in T cells with variable EBV-positive B cells (Song 2015).

How it differs from its parent: it is a spectrum from a virus-driven immunodeficiency disorder to a lymphoma, defined by grade; it spares nodes and marrow; and its low-grade forms are treated by restoring immune control (interferon alfa) rather than by chemotherapy.

How common: no registry figure in the sources read.

Treatment: on the National Cancer Institute approach, interferon alfa for grade 1 and 2 disease and rituximab-based chemotherapy (such as dose-adjusted EPOCH-R) for grade 3, with withdrawal of iatrogenic immunosuppression where present; relapse can move between grades (Song 2015 for the series behind the approach).

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: LYG; EBV-positive B-cell lymphoproliferative disorder of the lung; Angiocentric immunoproliferative lesion
- Tags: subtype-page; wave4; haematologic; rare
- Group: haematologic
- Burden: Rare: the National Cancer Institute reviewed 55 patients referred over 1995 to 2010, median age 46, men 2.2 times as often as women (Song 2015). No registry figure exists.
- Subtypes: Lymphomatoid granulomatosis, grade 1 and 2 (few EBV-positive large B cells; interferon); Lymphomatoid granulomatosis, grade 3 (overlaps EBV-positive large B-cell lymphoma; chemotherapy); Lymphomatoid granulomatosis with central nervous system involvement (38 percent)
- Biomarkers: EBV-encoded RNA in situ hybridisation in large B cells; Grade by number of EBV-positive B cells; Lung and brain imaging; Underlying immunodeficiency (iatrogenic, HIV, primary)

## 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/lymphomatoid-granulomatosis/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/lymphomatoid-granulomatosis/#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/lymphomatoid-granulomatosis/#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/lymphomatoid-granulomatosis/#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/lymphomatoid-granulomatosis/#treating-it [2 settings, 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/lymphomatoid-granulomatosis/#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/lymphomatoid-granulomatosis/#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/lymphomatoid-granulomatosis/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/lymphomatoid-granulomatosis/#living-with-it [10 questions, 3 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/lymphomatoid-granulomatosis/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/lymphomatoid-granulomatosis/data/ [15 connected records]

## Standard of care

- Grade 1 and 2: Interferon alfa, with withdrawal of iatrogenic immunosuppression. ([Interferon alfa-2a/2b](https://onco.cc/drugs/interferon-alfa/), [Non-Hodgkin lymphoma (all types)](https://onco.cc/cancers/non-hodgkin-lymphoma/))
- Grade 3: Rituximab-based chemotherapy as for large B-cell lymphoma. ([Rituximab](https://onco.cc/drugs/rituximab/), [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Diffuse large B-cell lymphoma](https://onco.cc/cancers/dlbcl/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Lymphomatoid_granulomatosis
- 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
- Katzenstein 2010, Am J Surg Pathol: lymphomatoid granulomatosis, insights over four decades: https://doi.org/10.1097/pas.0b013e3181fd8781
- Song 2015, Am J Surg Pathol: lymphomatoid granulomatosis, the National Cancer Institute series of 55 patients: https://doi.org/10.1097/pas.0000000000000328

## 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/), [Intravascular large B-cell lymphoma](https://onco.cc/cancers/intravascular-large-b-cell-lymphoma/), [Non-Hodgkin lymphoma (all types)](https://onco.cc/cancers/non-hodgkin-lymphoma/), [Post-transplant lymphoproliferative disorder (PTLD)](https://onco.cc/cancers/post-transplant-lymphoproliferative-disorder/)
- drugs: [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Interferon alfa-2a/2b](https://onco.cc/drugs/interferon-alfa/), [Rituximab](https://onco.cc/drugs/rituximab/)

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JSON: https://onco.cc/api/v1/entities/lymphomatoid-granulomatosis.json