{"entity":{"id":"t-cell-prolymphocytic-leukaemia","kind":"cancer","name":"T-cell prolymphocytic leukaemia","aka":["T-PLL","T-cell prolymphocytic leukemia","T-prolymphocytic leukaemia","T-cell chronic lymphocytic leukaemia (retired term)"],"tldr":"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).\n\nHow 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.\n\nHow common: no registry figure in the sources read.\n\nTreatment: 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).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/T-cell-prolymphocytic_leukemia","links":[{"label":"NCI PDQ: chronic lymphocytic leukaemia treatment","url":"https://www.cancer.gov/types/leukemia/patient/cll-treatment-pdq"},{"label":"Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"Staber 2019, Blood: consensus criteria for diagnosis, staging and response assessment of T-PLL","url":"https://doi.org/10.1182/blood.2019000402"},{"label":"Annals of Oncology 2017: characteristics, outcomes and treatment of 119 patients with T-PLL","url":"https://doi.org/10.1093/annonc/mdx163"},{"label":"Dearden 2011, Blood: alemtuzumab in T-PLL, intravenous against subcutaneous","url":"https://doi.org/10.1182/blood-2011-08-372854"}],"tags":["subtype-page","wave4","haematologic","rare"],"related":["leukaemia","cll","sezary-syndrome","t-large-granular-lymphocytic-leukaemia","splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli"],"cancers":[],"sections":[],"technologies":[],"targets":[],"drugs":["alemtuzumab","venetoclax","ibrutinib"],"companies":[],"institutions":[],"pathways":[],"terms":["allogeneic-transplant"],"trials":["nct04496349","nct02029443"],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"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)"],"standardOfCare":[{"setting":"Active disease","approach":"Intravenous alemtuzumab to best response, then allogeneic or autologous stem cell transplant in fit patients.","refs":["alemtuzumab","allogeneic-transplant","leukaemia"]},{"setting":"Relapsed","approach":"Venetoclax with or without ibrutinib; trials such as the MDM2 inhibitor study linked here.","refs":["venetoclax","ibrutinib","nct04496349"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"leukaemia"},"route":"/cancers/t-cell-prolymphocytic-leukaemia/","neighbours":{"cancer":[{"id":"cll","kind":"cancer","name":"Chronic lymphocytic leukaemia","route":"/cancers/cll/"},{"id":"leukaemia","kind":"cancer","name":"Leukaemia (all types)","route":"/cancers/leukaemia/"},{"id":"sezary-syndrome","kind":"cancer","name":"Sezary syndrome","route":"/cancers/sezary-syndrome/"},{"id":"splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli","kind":"cancer","name":"Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant)","route":"/cancers/splenic-b-cell-lymphoma-leukaemia-prominent-nucleoli/"},{"id":"t-large-granular-lymphocytic-leukaemia","kind":"cancer","name":"T-cell large granular lymphocytic leukaemia","route":"/cancers/t-large-granular-lymphocytic-leukaemia/"}],"drug":[{"id":"alemtuzumab","kind":"drug","name":"Alemtuzumab","route":"/drugs/alemtuzumab/"},{"id":"ibrutinib","kind":"drug","name":"Ibrutinib","route":"/drugs/ibrutinib/"},{"id":"venetoclax","kind":"drug","name":"Venetoclax","route":"/drugs/venetoclax/"}],"term":[{"id":"allogeneic-transplant","kind":"term","name":"Allogeneic stem cell transplant (allo-SCT)","route":"/terms/allogeneic-transplant/"}],"trial":[{"id":"nct04496349","kind":"trial","name":"A Study Evaluating APG-115 as a Single Agent or in Combination With APG-2575 in Subjects With R/R T-PLL and NHL","route":"/trials/nct04496349/"},{"id":"nct02029443","kind":"trial","name":"ACP-196 (Acalabrutinib), a Novel Bruton Tyrosine Kinase (BTK) Inhibitor, for Treatment of Chronic Lymphocytic Leukemia, Richter's Syndrome or Prolymphocytic Leukemia","route":"/trials/nct02029443/"}]}}