{"entity":{"id":"t-cell-histiocyte-rich-large-b-cell-lymphoma","kind":"cancer","name":"T-cell/histiocyte-rich large B-cell lymphoma","aka":["THRLBCL","T-cell/histiocyte-rich large B-cell lymphoma","T-cell-rich B-cell lymphoma","T-cell/histiocyte rich large B cell lymphoma"],"tldr":"A lymphoma in which the cancer cells are a tiny minority of what the pathologist sees: scattered large B cells in a dense crowd of normal T cells and macrophages. It is a form of large B-cell lymphoma, it usually presents with disease in the liver, spleen or bone marrow, and it is easy to mistake for a different disease in both directions.","summary":"What it is. A large B-cell lymphoma in which the malignant cells make up a very small fraction of the tissue. The rest is a reaction: sheets of normal T cells and histiocytes, the tissue form of the macrophage. The diagnosis therefore depends on recognising the scattered large B cells in a background that looks inflammatory, and on the pattern of markers they carry.\n\nHow it differs from its family. Ordinary diffuse large B-cell lymphoma is made of sheets of tumour cells. Here the tumour is outnumbered, and the clinical behaviour is different too: it tends to present at an advanced stage with the liver, the spleen and the bone marrow involved rather than with enlarged lymph nodes alone, and it affects men more often and at a younger age than most large B-cell lymphomas.\n\nThe boundary problem, which is the most important thing on this page. T-cell/histiocyte-rich large B-cell lymphoma sits at one end of a spectrum whose other end is nodular lymphocyte predominant Hodgkin lymphoma, a condition that usually behaves indolently. WHO-HAEM5 lists six growth patterns of nodular lymphocyte predominant Hodgkin lymphoma, and the last of them, pattern E, is described as diffuse and resembling this disease. The classification states plainly that in some cases a clear distinction may not be possible, and that it is especially difficult on a small biopsy. The International Consensus Classification renamed the Hodgkin disease nodular lymphocyte predominant B-cell lymphoma partly in recognition of that relationship.\n\nThe biology agrees that the boundary is soft. In a study that profiled gene expression in the tumour cells themselves, the three conditions did not cluster apart, and only a few genes were consistently different, and those only moderately. What differed was the surrounding tissue, the infiltrating T cells and histiocytes. That is an unusual situation in oncology: two diseases with different names, different stages at presentation and different treatments whose cancer cells look the same at the level of gene expression.\n\nHow it is treated. As a diffuse large B-cell lymphoma, with the same immunochemotherapy, which is a reasonable approach for a disease that behaves aggressively and carries CD20. The series are small and no randomised trial has been confined to it. The regimens are on the diffuse large B-cell lymphoma page and in the treatment layer of this family.","asOf":"2026-09-29","wikipedia":"https://en.wikipedia.org/wiki/Diffuse_large_B-cell_lymphoma","links":[{"label":"WHO Classification of Haematolymphoid Tumours, 5th edition: lymphoid neoplasms (Alaggio, Leukemia 2022)","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"International Consensus Classification of Mature Lymphoid Neoplasms (Campo, Blood 2022)","url":"https://doi.org/10.1182/blood.2022015851"},{"label":"Nodular lymphocyte predominant Hodgkin lymphoma and T-cell/histiocyte-rich large B-cell lymphoma, endpoints of a spectrum of one disease? (PLoS One 2013)","url":"https://doi.org/10.1371/journal.pone.0078812"},{"label":"Lymphoma incidence, survival and prevalence 2004 to 2014, subtype analyses from the UK Haematological Malignancy Research Network (Smith, Br J Cancer 2015)","url":"https://doi.org/10.1038/bjc.2015.94"}],"tags":["heme","lymphoma","subtype-page"],"related":["dlbcl","nodular-lymphocyte-predominant-hodgkin-lymphoma","hodgkin-lymphoma","non-hodgkin-lymphoma","mediastinal-grey-zone-lymphoma"],"cancers":[],"sections":[],"technologies":["histopathology-ihc","fdg-pet"],"targets":["cd20"],"drugs":[],"companies":[],"institutions":[],"pathways":[],"terms":["lymphoma-classification-2022","lymphoma-b-versus-t-cell","lymphoma-indolent-versus-aggressive","ipi-score"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"In the United Kingdom population series that reports lymphoma by subtype, 32 of 5,796 lymphomas (0.6 per cent) diagnosed between 2004 and 2012, a crude incidence of 0.30 and a European age-standardised rate of 0.10 per 100,000 a year, with a median age at diagnosis of 65.5 years. Five-year relative survival in that series was 67.9 per cent, with a wide confidence interval (46.8 to 82.0 per cent) because of the small number of patients.","subtypes":[],"biomarkers":["Scattered large B cells, fewer than about one in ten of the cells present, in a background of small T cells and histiocytes","The large cells express CD20 and BCL6; nuclear BCL6 was positive in 26 of 29 cases in one series","A background rich in CD8-positive T cells and CD68-positive histiocytes, which is part of the diagnosis rather than incidental","Absence of the nodular meshworks of follicular dendritic cells and of the small B cells that mark nodular lymphocyte predominant Hodgkin lymphoma","Epstein-Barr virus, which is characteristically negative"],"standardOfCare":[{"setting":"Making the diagnosis, and the two ways it goes wrong","approach":"Both errors are common and they point in opposite directions. Called inflammatory, the lymphoma is missed, because the tumour cells are a small minority and the tissue looks reactive. Called nodular lymphocyte predominant Hodgkin lymphoma, an aggressive disease is treated as an indolent one. WHO-HAEM5 states that a clear distinction from the diffuse pattern of that disease may not be possible in some cases, and that small biopsies are the hardest. A generous biopsy, read by a haematopathologist, with the surrounding cells examined as carefully as the tumour cells, is the answer the classification gives.","refs":["histopathology-ihc","nodular-lymphocyte-predominant-hodgkin-lymphoma","cd20","lymphoma-classification-2022"],"guideline":{"version":"WHO Classification of Haematolymphoid Tumours, 5th edition (2022), with the International Consensus Classification (2022) where they differ","url":"https://doi.org/10.1038/s41375-022-01620-2"}},{"setting":"Treatment","approach":"Treated as diffuse large B-cell lymphoma, with rituximab-containing immunochemotherapy, which is appropriate for a disease that carries CD20 and behaves aggressively. The staging usually finds advanced disease with the liver, spleen or bone marrow involved. No randomised trial has been confined to this entity; the regimens, the cycles and the evidence are on the diffuse large B-cell lymphoma page and in the treatment layer of this family.","refs":["dlbcl","rituximab","r-chop","lymphoma-tx-regimen-alphabet"],"guideline":{"version":"NCI PDQ: adult non-Hodgkin lymphoma treatment","url":"https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq"}}],"stateOfArt":[],"history":[{"year":2002,"title":"Recognised as heterogeneous and germinal-centre derived","note":"A review of 30 cases separated three appearances of the large cells and showed nuclear BCL6 in 26 of 29, placing the tumour cell in the germinal centre.","refs":[]},{"year":2013,"title":"Gene expression finds no clear line to the Hodgkin disease","note":"Profiling the microdissected tumour cells of nodular lymphocyte predominant Hodgkin lymphoma, its diffuse pattern and this disease found no consistent differences between them; the differences were in the surrounding T cells and histiocytes.","refs":["nodular-lymphocyte-predominant-hodgkin-lymphoma"]},{"year":2022,"title":"The relationship written into both classifications","note":"WHO-HAEM5 tabulates six growth patterns of nodular lymphocyte predominant Hodgkin lymphoma and names the diffuse one after this disease; the International Consensus Classification renamed that disease a B-cell lymphoma partly on the strength of the relationship.","refs":["lymphoma-classification-2022"]}],"pipeline":[],"openProblems":["The line between this disease and the diffuse pattern of nodular lymphocyte predominant Hodgkin lymphoma cannot always be drawn, and the two are treated differently.","The cancer cells of the two conditions are not distinguishable by gene expression. What differs is the immune cells around them, and nobody knows why the same tumour cell produces an indolent disease in one person and an aggressive one in another.","No trial has been confined to this entity, so the treatment is borrowed from diffuse large B-cell lymphoma on the strength of the shared surface marker."],"parent":"non-hodgkin-lymphoma"},"route":"/cancers/t-cell-histiocyte-rich-large-b-cell-lymphoma/","neighbours":{"cancer":[{"id":"dlbcl","kind":"cancer","name":"Diffuse large B-cell lymphoma","route":"/cancers/dlbcl/"},{"id":"hodgkin-lymphoma","kind":"cancer","name":"Hodgkin lymphoma","route":"/cancers/hodgkin-lymphoma/"},{"id":"mediastinal-grey-zone-lymphoma","kind":"cancer","name":"Mediastinal grey zone lymphoma","route":"/cancers/mediastinal-grey-zone-lymphoma/"},{"id":"nodular-lymphocyte-predominant-hodgkin-lymphoma","kind":"cancer","name":"Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma)","route":"/cancers/nodular-lymphocyte-predominant-hodgkin-lymphoma/"},{"id":"non-hodgkin-lymphoma","kind":"cancer","name":"Non-Hodgkin lymphoma (all types)","route":"/cancers/non-hodgkin-lymphoma/"}],"technology":[{"id":"fdg-pet","kind":"technology","name":"FDG PET","route":"/technologies/fdg-pet/"},{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"}],"target":[{"id":"cd20","kind":"target","name":"CD20","route":"/targets/cd20/"}],"term":[{"id":"lymphoma-b-versus-t-cell","kind":"term","name":"B-cell, T-cell and NK-cell lymphoma","route":"/terms/lymphoma-b-versus-t-cell/"},{"id":"lymphoma-indolent-versus-aggressive","kind":"term","name":"Indolent and aggressive lymphoma","route":"/terms/lymphoma-indolent-versus-aggressive/"},{"id":"ipi-score","kind":"term","name":"International Prognostic Index (IPI)","route":"/terms/ipi-score/"},{"id":"r-chop","kind":"term","name":"R-CHOP (lymphoma chemoimmunotherapy)","route":"/terms/r-chop/"},{"id":"lymphoma-tx-regimen-alphabet","kind":"term","name":"The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest","route":"/terms/lymphoma-tx-regimen-alphabet/"},{"id":"lymphoma-classification-2022","kind":"term","name":"The two lymphoma classifications of 2022 (WHO-HAEM5 and ICC)","route":"/terms/lymphoma-classification-2022/"}],"drug":[{"id":"rituximab","kind":"drug","name":"Rituximab","route":"/drugs/rituximab/"}]}}