Loading
6 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
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.
| Setting | Approach | Guideline |
|---|---|---|
| Active disease | Intravenous alemtuzumab to best response, then allogeneic or autologous stem cell transplant in fit patients. | not mapped |
| Relapsed | Venetoclax with or without ibrutinib; trials such as the MDM2 inhibitor study linked here. | not mapped |