Loading
T-cell prolymphocytic leukaemia is a rare, aggressive leukaemia of mature T cells in older adults, with a very high white cell count, a big spleen and liver, swollen nodes and sometimes skin changes. The antibody alemtuzumab given into a vein clears it in most people, but it returns within a year or two unless a stem cell transplant is done in remission.
WHO-HAEM5 keeps T-cell prolymphocytic leukaemia as a mature T-cell leukaemia defined by inversion or translocation of chromosome 14 activating TCL1A (or MTCP1 on the X chromosome), with a small-cell variant that was once called T-cell chronic lymphocytic leukaemia (Alaggio 2022). The T-PLL International Study Group's 2019 consensus set standard criteria for diagnosis, treatment indication and response assessment so that trials can be compared (Staber 2019). In 119 patients at one centre, complex karyotype was present in 65 percent and chromosome 14 aberrations in 52 percent, 80 percent had died at analysis and median overall survival from diagnosis was 19 months (Annals of Oncology 2017). Intravenous alemtuzumab as first-line treatment gave an overall response of 91 percent with 81 percent complete responses in 32 patients, while the subcutaneous route responded in only 3 of 9 and was abandoned (Dearden 2011).
How it differs from its parent: the leukaemia page groups the leukaemias; this is a mature T-cell disease with a specific chromosome 14 lesion, resistant to conventional chemotherapy and dependent on a single antibody and transplant, and it can be inactive for months before accelerating, so the consensus criteria define when to treat.
How common: no registry figure in the sources read.
Treatment: intravenous alemtuzumab to best response, then allogeneic (or autologous) stem cell transplant in fit patients, because remissions without consolidation are short; pentostatin with alemtuzumab, and venetoclax with or without ibrutinib or the MDM2 inhibitor trial linked here, for relapsed disease (Staber 2019; Dearden 2011).
Rare: the largest single-centre series holds 119 consecutive patients seen between 1990 and 2016 (Annals of Oncology 2017). 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, 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
Intravenous alemtuzumab to best response, then allogeneic or autologous stem cell transplant in fit patients.
Venetoclax with or without ibrutinib; trials such as the MDM2 inhibitor study linked here.
Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.
Query for this cancer: (TITLE:"T-cell prolymphocytic leukaemia" OR ABSTRACT:"T-cell prolymphocytic leukaemia" OR TITLE:"T-PLL" OR ABSTRACT:"T-PLL" OR TITLE:"T-cell prolymphocytic leukemia" OR ABSTRACT:"T-cell prolymphocytic leukemia" OR TITLE:"T-prolymphocytic leukaemia" OR ABSTRACT:"T-prolymphocytic leukaemia" OR TITLE:"T-cell chronic lymphocytic leukaemia retired term" OR ABSTRACT:"T-cell chronic lymphocytic leukaemia retired term") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about T-cell prolymphocytic leukaemia, not a curated reading list.
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, and the expert centres with trials on record.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Blood in stool or urine, vomiting blood, a bleed that will not stop, or a severe headache; fatal bleeding events have occurred and the labels advise considering the risk around surgery and with blood thinners.
Nausea, vomiting, muscle cramps, palpitations, seizures, confusion or passing much less urine in the first days of a new dose; the label requires hydration, anti-hyperuricaemic drugs and blood tests around each ramp-up step.
Avoid grapefruit and Seville oranges.
Take with a meal and water. Avoid grapefruit, Seville oranges and starfruit.
Possible QT prolongation. Check ECG and electrolytes; review other QT-prolonging drugs.
140 mg daily (mild), 70 mg (moderate); avoid in severe.
See all on the product pages:IbrutinibVenetoclax·Printable cards in the navigator
Newly diagnosed? Read the first 60 days with T-cell prolymphocytic leukaemia, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record.
Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked.