T-cell prolymphocytic leukemia

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*We have moved How I Treat articles to a dedicated page.
Last updated on 2024-07-23:5 regimens on this page
5 variants on this page
=Guidelines=
Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.
==NCCN==
*NCCN does not currently have guidelines at this granular level; please see NCCN Guidelines - T-cell Lymphomas.
=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 	Dates of enrollment	Evidence 	Efficacy
Dearden et al. 2001 	1993-03 to 2000-05 	Non-randomized	ORR: 76%, CR: 60%
Dearden et al. 2011 (UKCLL05)	2005-10 to 2007-07 	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 dosing details in manuscript have been reviewed by our editors EuroPMC
#UKCLL05: 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 dosing details in manuscript have been reviewed by our editors EuroPMC EudraCT 2004-004636-31
==Pentostatin & Alemtuzumab==
===Regimen===
Study 	Dates of enrollment	Evidence 	Efficacy
Ravandi et al. 2009 (MDACC 2004-0408)	2004-10 to 2008-05 	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 dosing details in manuscript have been reviewed by our editors EuroPMC
=Relapsed or refractory, salvage therapy=
==Bendamustine monotherapy==
===Regimen===
Study 	Dates of enrollment	Evidence
Herbaux et al. 2014	2009-2013 	Retrospective
====Chemotherapy====
* Bendamustine 70 to 120 mg/m2 IV over 30 to 60 minutes once per day on days 1 & 2 
21-day cycle for 6 cycles
===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. Epub 2014 Oct 15. link to original article dosing details in manuscript have been reviewed by our editors EuroPMC
==Pentostatin & Alemtuzumab==
===Regimen===
Study 	Dates of enrollment	Evidence 	Efficacy
Ravandi et al. 2009 (MDACC 2004-0408)	2004-10 to 2008-05 	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 EuroPMC
==Ibrutinib & Venetoclax==
===Regimen===
Study 	Evidence
Kornauth et al. 2019	Case series
====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. 
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===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 EuroPMC
# 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 EuroPMC
# 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 EuroPMC
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