# T-cell prolymphocytic leukaemia

Source: https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/  
OnCo record `t-cell-prolymphocytic-leukaemia` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

## Summary

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).

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: T-PLL; T-cell prolymphocytic leukemia; T-prolymphocytic leukaemia; T-cell chronic lymphocytic leukaemia (retired term)
- Tags: subtype-page; wave4; haematologic; rare
- Group: haematologic
- Burden: 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.
- Subtypes: T-cell prolymphocytic leukaemia, typical (TCL1A rearrangement); Small-cell variant of T-PLL (about a fifth of cases; formerly T-CLL); Cerebriform variant of T-PLL; Inactive T-PLL (watch until the consensus treatment criteria are met)
- Biomarkers: inv(14) or t(14;14) with TCL1A expression, or MTCP1 rearrangement; Complex karyotype and ATM mutations; CD52 expression (alemtuzumab target); Lymphocyte count and doubling time (treatment indication)

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/t-cell-prolymphocytic-leukaemia/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/#what-it-is [4 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/#treating-it [2 settings, 1 decision with options]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/#evidence [2 trials]
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/#science [3 targets]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/#living-with-it [11 questions, 6 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/coming/ [3 medicines]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/t-cell-prolymphocytic-leukaemia/data/ [16 connected records]

## Standard of care

- Active disease: Intravenous alemtuzumab to best response, then allogeneic or autologous stem cell transplant in fit patients. ([Alemtuzumab](https://onco.cc/drugs/alemtuzumab/), [Allogeneic stem cell transplant (allo-SCT)](https://onco.cc/terms/allogeneic-transplant/), [Leukaemia (all types)](https://onco.cc/cancers/leukaemia/))
- Relapsed: Venetoclax with or without ibrutinib; trials such as the MDM2 inhibitor study linked here. ([Venetoclax](https://onco.cc/drugs/venetoclax/), [Ibrutinib](https://onco.cc/drugs/ibrutinib/), [A Study Evaluating APG-115 as a Single Agent or in Combination With APG-2575 in Subjects With R/R T-PLL and NHL](https://onco.cc/trials/nct04496349/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/T-cell-prolymphocytic_leukemia
- NCI PDQ: chronic lymphocytic leukaemia treatment: https://www.cancer.gov/types/leukemia/patient/cll-treatment-pdq
- Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms: https://doi.org/10.1038/s41375-022-01620-2
- Staber 2019, Blood: consensus criteria for diagnosis, staging and response assessment of T-PLL: https://doi.org/10.1182/blood.2019000402
- Annals of Oncology 2017: characteristics, outcomes and treatment of 119 patients with T-PLL: https://doi.org/10.1093/annonc/mdx163
- Dearden 2011, Blood: alemtuzumab in T-PLL, intravenous against subcutaneous: https://doi.org/10.1182/blood-2011-08-372854

## Connected records

- cancers: [Chronic lymphocytic leukaemia](https://onco.cc/cancers/cll/), [Leukaemia (all types)](https://onco.cc/cancers/leukaemia/), [Sezary syndrome](https://onco.cc/cancers/sezary-syndrome/), [Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant)](https://onco.cc/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/), [T-cell large granular lymphocytic leukaemia](https://onco.cc/cancers/t-large-granular-lymphocytic-leukaemia/)
- drugs: [Alemtuzumab](https://onco.cc/drugs/alemtuzumab/), [Ibrutinib](https://onco.cc/drugs/ibrutinib/), [Venetoclax](https://onco.cc/drugs/venetoclax/)
- terms: [Allogeneic stem cell transplant (allo-SCT)](https://onco.cc/terms/allogeneic-transplant/)
- trials: [A Study Evaluating APG-115 as a Single Agent or in Combination With APG-2575 in Subjects With R/R T-PLL and NHL](https://onco.cc/trials/nct04496349/), [ACP-196 (Acalabrutinib), a Novel Bruton Tyrosine Kinase (BTK) Inhibitor, for Treatment of Chronic Lymphocytic Leukemia, Richter's Syndrome or Prolymphocytic Leukemia](https://onco.cc/trials/nct02029443/)

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JSON: https://onco.cc/api/v1/entities/t-cell-prolymphocytic-leukaemia.json