{"entity":{"id":"intravascular-large-b-cell-lymphoma","kind":"cancer","name":"Intravascular large B-cell lymphoma","aka":["IVLBCL","Intravascular lymphoma","Angiotropic large cell lymphoma","Malignant angioendotheliomatosis"],"tldr":"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).\n\nHow 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.\n\nHow common: no registry figure; 182 published cases in a decade (Cancer Management and Research 2020).\n\nTreatment: 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.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Intravascular_lymphomas","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":"PRIMEUR-IVL (Shimada 2020, Lancet Oncology): R-CHOP with high-dose methotrexate and intrathecal chemotherapy in intravascular large B-cell lymphoma","url":"https://doi.org/10.1016/s1470-2045(20)30059-0"},{"label":"Cancer Management and Research 2020: prognosis of intravascular large B-cell lymphoma, 182 published cases","url":"https://doi.org/10.2147/cmar.s267825"}],"tags":["subtype-page","wave4","haematologic","rare"],"related":["dlbcl","primary-cns-lymphoma","lymphomatoid-granulomatosis","hiv-associated-lymphoma"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":["rituximab","cyclophosphamide","methotrexate"],"companies":[],"institutions":[],"pathways":[],"terms":["r-chop","myd88-l265p"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"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)"],"standardOfCare":[{"setting":"All cases","approach":"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.","refs":["rituximab","cyclophosphamide","methotrexate","r-chop","primary-cns-lymphoma"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"dlbcl"},"route":"/cancers/intravascular-large-b-cell-lymphoma/","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":"lymphomatoid-granulomatosis","kind":"cancer","name":"Lymphomatoid granulomatosis","route":"/cancers/lymphomatoid-granulomatosis/"},{"id":"primary-cns-lymphoma","kind":"cancer","name":"Primary CNS lymphoma","route":"/cancers/primary-cns-lymphoma/"}],"drug":[{"id":"cyclophosphamide","kind":"drug","name":"Cyclophosphamide","route":"/drugs/cyclophosphamide/"},{"id":"methotrexate","kind":"drug","name":"Methotrexate","route":"/drugs/methotrexate/"},{"id":"rituximab","kind":"drug","name":"Rituximab","route":"/drugs/rituximab/"}],"term":[{"id":"myd88-l265p","kind":"term","name":"MYD88 L265P and CXCR4 mutations","route":"/terms/myd88-l265p/"},{"id":"r-chop","kind":"term","name":"R-CHOP (lymphoma chemoimmunotherapy)","route":"/terms/r-chop/"}]}}