T-cell prolymphocytic leukemia

Section editor
	Bhagirathbhai Dholaria, MBBS Vanderbilt University Nashville, TN

5 regimens on this page 5 variants on this page

Guidelines

NCCN

• 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

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Regimen

Study	Evidence	Efficacy
Dearden et al. 2001	Non-randomized	ORR: 76%, CR: 60%
Dearden et al. 2011	Non-randomized	ORR: 91%, CR: 81%

Chemotherapy

• Alemtuzumab (Campath)

First week: 3 mg IV on the first day,10 mg IV on the second day and 30 mg IV on the third day. Subsequent weeks: 30 mg IV three times weekly until achievement of CR or best response or for up to a total of 3 months

Alemtuzumab, Pentostatin

Regimen

Study	Evidence	Efficacy
Ravandi et al. 2009	Non-randomized	ORR: 69%, CR: 62%

Chemotherapy

• Alemtuzumab (Campath) First week: 3 mg IV on the first day,10 mg IV on the second day and 30 mg IV on the third day. Subsequent weeks: 30 mg IV three times weekly until achievement of CR or best response or for up to a total of 3 months
• Pentostatin (Nipent) 4 mg/m² IV weekly for 4 weeks then every 2 weeks until they achieved a CR or best response or for up to 10 more doses (total of 14 doses)

References

1. 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
2. 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
3. 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. link to original article PubMed

Relapsed or refractory, salvage therapy

Alemtuzumab, Pentostatin

Regimen

Study	Evidence	Efficacy
Ravandi et al. 2009	Non-randomized	ORR: 69%, CR: 62%

Chemotherapy

• Alemtuzumab (Campath) First week: 3 mg IV on the first day,10 mg IV on the second day and 30 mg IV on the third day. Subsequent weeks: 30 mg IV three times weekly until achievement of CR or best response or for up to a total of 3 months
• Pentostatin (Nipent) 4 mg/m² IV weekly for 4 weeks then every 2 weeks until they achieved a CR or best response or for up to 10 more doses (total of 14 doses)

References

1. 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. link to original article PubMed

Bendamustine

Regimen

Study	Evidence
Herbaux et al. 2014	Retrospective

• Bendamustine 70 to 120 mg/m² IV, days 1 and 2 every 3 weeks for total 6 cycles

References

1. 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
   

Venetoclax, Ibrutinib (ongoing clinical trial, NCT03873493)

Regimen

• Venetoclax 400 mg, potentially up to 600 mg, orally once daily
• Ibrutinib 420 mg dosed orally once daily

References

1. 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 original article

Consolidation therapy after upfront or salvage therapy

Allogeneic HSCT evaluation suggested in eligible patients.

References

1. 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
2. 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 PubMed
3. 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