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T-cell large granular lymphocytic leukaemia is a slow, usually non-fatal leukaemia in which a clone of cytotoxic T cells builds up in the blood and marrow and turns the immune system against the body, causing low neutrophil counts, anaemia and often rheumatoid arthritis. It is treated only when it causes problems, with low-dose immune-suppressing drugs rather than chemotherapy.
WHO-HAEM5 keeps T-cell large granular lymphocytic leukaemia as a mature T-cell leukaemia of clonal CD3-positive CD8-positive cytotoxic lymphocytes, beside the related chronic lymphoproliferative disorder of NK cells (Alaggio 2022). Exome sequencing found somatic STAT3 mutations in 31 of 77 patients (40 percent), all in the SH2 domain (Y640F 17 percent, D661V and D661Y 9 percent each, N647I 4 percent), causing STAT3 phosphorylation and nuclear localisation; the disease is often associated with autoimmune disorders and immune-mediated cytopenias (Koskela 2012). In 204 patients, therapy was needed mostly for anaemia and neutropenia; methotrexate, cyclosporin and cyclophosphamide each gave overall responses of 40 to 50 percent, sequential use responded in most, only 10 to 20 percent needed salvage (antithymocyte globulin, alemtuzumab, tofacitinib, splenectomy or abatacept), methotrexate gave the most durable responses, and STAT3-mutated patients needed therapy more often but had better overall survival (Leukemia and Lymphoma 2018).
How it differs from its parent: it is indolent and immune-mediated rather than proliferative; its harm comes from cytopenias and autoimmunity, not from tumour bulk; and its treatment is immunosuppression, with JAK-STAT inhibition the rational new direction from the STAT3 findings.
How common: no registry figure in the sources read.
Treatment: observation without cytopenias or symptoms; low-dose oral methotrexate, cyclosporin or cyclophosphamide when treatment is indicated, used sequentially; salvage with alemtuzumab, JAK inhibitors, splenectomy or abatacept; the PI3K-delta inhibitor and anti-CD94 antibody trials linked here (Leukemia and Lymphoma 2018).
Rare and chronic; the largest treatment series holds 204 patients meeting uniform criteria, followed for a median of 36 months (Leukemia and Lymphoma 2018). No registry figure is in the sources read.
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: 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), 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
Low-dose methotrexate, cyclosporin or cyclophosphamide, used sequentially; salvage with alemtuzumab, JAK inhibitors or splenectomy.
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Query for this cancer: (TITLE:"T-cell large granular lymphocytic leukaemia" OR ABSTRACT:"T-cell large granular lymphocytic leukaemia" OR TITLE:"Large granular lymphocytic leukemia" OR ABSTRACT:"Large granular lymphocytic leukemia" OR TITLE:"Large granular lymphocytic leukaemia" OR ABSTRACT:"Large granular lymphocytic leukaemia" OR TITLE:"LGL leukaemia" OR ABSTRACT:"LGL leukaemia" OR TITLE:"T-LGL leukaemia" OR ABSTRACT:"T-LGL leukaemia" OR TITLE:"T-LGLL" OR ABSTRACT:"T-LGLL") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about T-cell large granular lymphocytic leukaemia, not a curated reading list.
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Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
High-dose methotrexate requires normal renal function, hydration, urine alkalinisation and leucovorin rescue with level monitoring.
Reduce dose for CrCl below 60 with platelets under 150; dialysis dosing after each session.
Intense itching, prickling or burning of the skin within minutes of contact with water, typically after a shower. It affects a large minority of people with polycythaemia vera and can be the most disabling symptom.
See all on the product pages:CyclophosphamideMethotrexateRuxolitinib·Printable cards in the navigator
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