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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.
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.
How 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.
The 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.
The 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.
How 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.
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.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, Plasmablastic lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Ocular adnexal MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Lymphomatoid papulosis, Mycosis fungoides, Enteropathy-associated T-cell lymphoma, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
No subtypes recorded beyond the ones named in the family strip above.
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.
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.
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Query for this cancer: (TITLE:"T-cell/histiocyte-rich large B-cell lymphoma" OR ABSTRACT:"T-cell/histiocyte-rich large B-cell lymphoma" OR TITLE:"THRLBCL" OR ABSTRACT:"THRLBCL" OR TITLE:"T-cell-rich B-cell lymphoma" OR ABSTRACT:"T-cell-rich B-cell lymphoma" OR TITLE:"T-cell/histiocyte rich large B cell lymphoma" OR ABSTRACT:"T-cell/histiocyte rich large B cell lymphoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about T-cell/histiocyte-rich large B-cell lymphoma, not a curated reading list.
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.
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.
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.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Anyone who has ever had hepatitis B can have the virus wake up when rituximab strips out their B cells, sometimes months later and sometimes fatally. A blood test before the first dose, and a tablet for those who need it, prevents almost all of it.
A shortage of antibodies because treatment has wiped out the B cells or plasma cells that make them, as CD19 CAR-T, CD20 antibodies and BCMA therapies deliberately do. It leaves patients prone to infections, sometimes for years, and is managed with immunoglobulin infusions.
Treatments that remove B cells also remove the antibodies B cells make, and some people never make them again. When repeated chest and sinus infections follow, donated antibody given monthly by drip or weekly under the skin prevents them.
Reactions around the moment a drug is given: chills, fever or breathlessness from antibodies (infusion reactions), true allergy (hypersensitivity, rarely anaphylaxis), and leakage of a damaging drug into tissue around the vein (extravasation).
See all on the product pages:Rituximab·Printable cards in the navigator
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