{"entity":{"id":"lymphomatoid-granulomatosis","kind":"cancer","name":"Lymphomatoid granulomatosis","aka":["LYG","EBV-positive B-cell lymphoproliferative disorder of the lung","Angiocentric immunoproliferative lesion"],"tldr":"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).\n\nHow 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.\n\nHow common: no registry figure in the sources read.\n\nTreatment: 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).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Lymphomatoid_granulomatosis","links":[{"label":"NCI PDQ: adult non-Hodgkin lymphoma treatment","url":"https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq"},{"label":"Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"Katzenstein 2010, Am J Surg Pathol: lymphomatoid granulomatosis, insights over four decades","url":"https://doi.org/10.1097/pas.0b013e3181fd8781"},{"label":"Song 2015, Am J Surg Pathol: lymphomatoid granulomatosis, the National Cancer Institute series of 55 patients","url":"https://doi.org/10.1097/pas.0000000000000328"}],"tags":["subtype-page","wave4","haematologic","rare"],"related":["non-hodgkin-lymphoma","dlbcl","intravascular-large-b-cell-lymphoma","post-transplant-lymphoproliferative-disorder","hiv-associated-lymphoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":["interferon-alfa","rituximab","cyclophosphamide"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"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)"],"standardOfCare":[{"setting":"Grade 1 and 2","approach":"Interferon alfa, with withdrawal of iatrogenic immunosuppression.","refs":["interferon-alfa","non-hodgkin-lymphoma"]},{"setting":"Grade 3","approach":"Rituximab-based chemotherapy as for large B-cell lymphoma.","refs":["rituximab","cyclophosphamide","dlbcl"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"non-hodgkin-lymphoma"},"route":"/cancers/lymphomatoid-granulomatosis/","neighbours":{"cancer":[{"id":"dlbcl","kind":"cancer","name":"Diffuse large B-cell lymphoma","route":"/cancers/dlbcl/"},{"id":"hiv-associated-lymphoma","kind":"cancer","name":"HIV-associated (AIDS-related) lymphomas","route":"/cancers/hiv-associated-lymphoma/"},{"id":"intravascular-large-b-cell-lymphoma","kind":"cancer","name":"Intravascular large B-cell lymphoma","route":"/cancers/intravascular-large-b-cell-lymphoma/"},{"id":"non-hodgkin-lymphoma","kind":"cancer","name":"Non-Hodgkin lymphoma (all types)","route":"/cancers/non-hodgkin-lymphoma/"},{"id":"post-transplant-lymphoproliferative-disorder","kind":"cancer","name":"Post-transplant lymphoproliferative disorder (PTLD)","route":"/cancers/post-transplant-lymphoproliferative-disorder/"}],"drug":[{"id":"cyclophosphamide","kind":"drug","name":"Cyclophosphamide","route":"/drugs/cyclophosphamide/"},{"id":"interferon-alfa","kind":"drug","name":"Interferon alfa-2a/2b","route":"/drugs/interferon-alfa/"},{"id":"rituximab","kind":"drug","name":"Rituximab","route":"/drugs/rituximab/"}]}}