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Guidelines
"How I Treat"
- 2012: Dearden How I treat prolymphocytic leukemia
NCCN
Diagnosis, staging and treatment response criteria (TPLL-ISG)
- 2019: Staber et al. Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia
Upfront induction therapy
Alemtuzumab monotherapy
Regimen
| Study | Evidence | Efficacy |
|---|---|---|
| Dearden et al. 2001 | Non-randomized | ORR: 76%, CR: 60% |
| Dearden et al. 2011 | Non-randomized | ORR: 91%, CR: 81% |
Targeted therapy
- Alemtuzumab (Campath) as follows:
- Week 1: 3 mg IV once on day 1, then 10 mg IV once on day 2, then 30 mg IV once on day 3
- Week 2 onwards: 30 mg IV three times weekly
Continued until achievement of CR or best response or for up to a total of 3 months
References
- Dearden CE, Matutes E, Cazin B, Tjønnfjord GE, Parreira A, Nomdedeu B, Leoni P, Clark FJ, Radia D, Rassam SM, Roques T, Ketterer N, Brito-Babapulle V, Dyer MJ, Catovsky D. High remission rate in T-cell prolymphocytic leukemia with CAMPATH-1H. Blood. 2001 Sep 15;98(6):1721-6. link to original article PubMed
- Dearden CE, Khot A, Else M, Hamblin M, Grand E, Roy A, Hewamana S, Matutes E, Catovsky D. Alemtuzumab therapy in T-cell prolymphocytic leukemia: comparing efficacy in a series treated intravenously and a study piloting the subcutaneous route. Blood. 2011 Nov 24;118(22):5799-802. Epub 2011 Sep 26. link to original article PubMed
Pentostatin & Alemtuzumab
Regimen
| Study | Evidence | Efficacy |
|---|---|---|
| Ravandi et al. 2009 (MDACC 2004-0408) | Non-randomized | ORR: 69%, CR: 62% |
Targeted therapy
- Alemtuzumab (Campath) as follows:
- Week 1: 3 mg IV once on day 1, then 10 mg IV once on day 2, then 30 mg IV once on day 3
- Week 2 onwards: 30 mg IV three times weekly
Chemotherapy
- Pentostatin (Nipent) as follows:
- Weeks 1 to 4: 4 mg/m2 IV once per week
- Week 5 onwards: 4 mg/m2 IV once every 2 weeks
Continued until achievement of CR or best response or for up to a total of 3 months (total of 14 doses of pentostatin)
References
- MDACC 2004-0408: Ravandi F, Aribi A, O'Brien S, Faderl S, Jones D, Ferrajoli A, Huang X, York S, Pierce S, Wierda W, Kontoyiannis D, Verstovsek S, Pro B, Fayad L, Keating M, Kantarjian H. Phase II study of alemtuzumab in combination with pentostatin in patients with T-cell neoplasms. J Clin Oncol. 2009 Nov 10;27(32):5425-30. Epub 2009 Oct 5. link to original article link to PMC article PubMed
Relapsed or refractory, salvage therapy
Bendamustine monotherapy
Regimen
| Study | Evidence |
|---|---|
| Herbaux et al. 2014 | Retrospective |
References
- Retrospective: Herbaux C, Genet P, Bouabdallah K, Pignon JM, Debarri H, Guidez S, Betrian S, Leleu X, Facon T, Morschhauser F, Damaj G, Cazin B, Ysebaert L. Bendamustine is effective in T-cell prolymphocytic leukaemia. Br J Haematol. 2015 Mar;168(6):916-9. link to original article PubMed
Pentostatin & Alemtuzumab
Regimen
| Study | Evidence | Efficacy |
|---|---|---|
| Ravandi et al. 2009 (MDACC 2004-0408) | Non-randomized | ORR: 69%, CR: 62% |
Targeted therapy
- Alemtuzumab (Campath) as follows:
- Week 1: 3 mg IV once on day 1, then 10 mg IV once on day 2, then 30 mg IV once on day 3
- Week 2 onwards: 30 mg IV three times weekly
Chemotherapy
- Pentostatin (Nipent) as follows:
- Weeks 1 to 4: 4 mg/m2 IV once per week
- Week 5 onwards: 4 mg/m2 IV once every 2 weeks
Continued until achievement of CR or best response or for up to a total of 3 months (total of 14 doses of pentostatin)
References
- MDACC 2004-0408: Ravandi F, Aribi A, O'Brien S, Faderl S, Jones D, Ferrajoli A, Huang X, York S, Pierce S, Wierda W, Kontoyiannis D, Verstovsek S, Pro B, Fayad L, Keating M, Kantarjian H. Phase II study of alemtuzumab in combination with pentostatin in patients with T-cell neoplasms. J Clin Oncol. 2009 Nov 10;27(32):5425-30. Epub 2009 Oct 5. link to original article link to PMC article PubMed
Ibrutinib & Venetoclax
Regimen
| Study | Evidence |
|---|---|
| Kornauth et al. 2019 | Non-randomized |
Targeted therapy
- Ibrutinib 420 mg PO once per day
- Venetoclax 400 to 600 mg PO once per day
Continued indefinitely
References
- Abstract: Kornauth C, Herbaux C, Boidol B, Guillemette C, Caron P, Poulain S, et al. Combination of Venetoclax and Ibrutinib Increases bcl2-Dependent Apoptotic Priming, Reduces ITK-Phosphorylation and Is Clinically Promising in Relapsed/Refractory T-Prolymphocytic Leukemia. Blood. 2019;134(Supplement_1):3965. link to abstract
Consolidation therapy after upfront or salvage therapy
Allogeneic HSCT evaluation suggested in eligible patients.
References
- Krishnan B, Else M, Tjonnfjord GE, Cazin B, Carney D, Carter J, Ketterer N, Catovsky D, Ethell M, Matutes E, Dearden CE. Stem cell transplantation after alemtuzumab in T-cell prolymphocytic leukaemia results in longer survival than after alemtuzumab alone: a multicentre retrospective study. Br J Haematol. 2010 Jun;149(6):907-10. link to original article PubMed
- Kalaycio ME, Kukreja M, Woolfrey AE, Szer J, Cortes J, Maziarz RT, Bolwell BJ, Buser A, Copelan E, Gale RP, Gupta V, Maharaj D, Marks DI, Pavletic SZ, Horowitz MM, Arora M. Allogeneic hematopoietic cell transplant for prolymphocytic leukemia. Biol Blood Marrow Transplant. 2010 Apr;16(4):543-7. link to original article link to PMC article PubMed
- Retrospective: Wiktor-Jedrzejczak W, Dearden C, de Wreede L, van Biezen A, Brinch L, Leblond V, Brune M, Volin L, Kazmi M, Nagler A, Schetelig J, de Witte T, Dreger P; EBMT Chronic Leukemia Working Party. Hematopoietic stem cell transplantation in T-prolymphocytic leukemia: a retrospective study from the European Group for Blood and Marrow Transplantation and the Royal Marsden Consortium. Leukemia. 2012 May;26(5):972-6. link to original article PubMed