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A slow-growing lymphoma can change into a fast-growing one. It usually announces itself as one node growing much faster than the others, a sudden rise in lactate dehydrogenase, or new fevers and weight loss in somebody who has been stable for years. It is treated as the aggressive lymphoma it has become, not as the one it came from.
WHO-HAEM5 made transformations of indolent B-cell lymphomas a family in its own right in 2022, which they had never been before. The commonest route is follicular lymphoma to diffuse large B-cell lymphoma; chronic lymphocytic leukaemia to diffuse large B-cell lymphoma is called Richter transformation and has its own page; marginal zone and lymphoplasmacytic lymphomas transform less often.
How it shows itself: one site growing out of proportion to the rest, a rapidly rising lactate dehydrogenase, new B symptoms, hypercalcaemia, or a new area of high uptake on a PET scan against a background of low uptake. A biopsy of the most active site, chosen on the PET scan, is what confirms it, and biopsying the wrong node is the commonest way to miss it.
Why it matters. Transformation is the commonest cause of death in follicular lymphoma, and it changes the treatment completely: the disease is treated as an aggressive lymphoma, with combination immunochemotherapy and, in people who have already had chemotherapy for the indolent disease, consideration of CAR-T or transplant. The outcome depends heavily on whether the person has had chemotherapy before.
What is not known. There is no marker that identifies in advance which indolent lymphoma will transform, and no treatment that has been shown to prevent it. Treating an indolent lymphoma earlier does not reduce the risk.
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, T-cell/histiocyte-rich large B-cell 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, 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
Showing the organ this term concerns: Follicular lymphoma.
Shares Gastric MALT lymphoma, Marginal zone lymphoma, Non-Hodgkin lymphoma (all types) and the tags heme, lymphoma.
Shares Indolent and aggressive lymphoma, Gastric MALT lymphoma, Mycosis fungoides, Diffuse large B-cell lymphoma and the tags heme, lymphoma.
Shares Gastric MALT lymphoma, Mycosis fungoides, Marginal zone lymphoma, Non-Hodgkin lymphoma (all types) and the tags heme, lymphoma.
Shares Indolent and aggressive lymphoma, Gastric MALT lymphoma, Watch and wait in lymphoma: when the right treatment is none yet, Marginal zone lymphoma and the tags heme, lymphoma.
Shares Diffuse large B-cell lymphoma, Non-Hodgkin lymphoma (all types) and the tags heme, lymphoma.
Shares Non-Hodgkin lymphoma (all types) and the tags heme, lymphoma.
Shares Non-Hodgkin lymphoma (all types) and the tags heme, lymphoma.
Shares Indolent and aggressive lymphoma, Watch and wait in lymphoma: when the right treatment is none yet, Mycosis fungoides and the tags heme, lymphoma.