T-cell prolymphocytic leukemia

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Bhagirathbhai Dholaria, MBBS
Vanderbilt University
Nashville, TN
5 regimens on this page
5 variants on this page


Guidelines

NCCN

Diagnosis, staging and treatment response criteria (TPLL-ISG)

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) as follows:
    • First week: 3 mg IV once on day 1, then 10 mg IV once on day 2, then 30 mg IV once on day 3
    • Subsequent weeks: 30 mg IV three times weekly

Continued until achievement of CR or best response or for up to a total of 3 months

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

Pentostatin & Alemtuzumab

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Regimen

Study Evidence Efficacy
Ravandi et al. 2009 (MDACC 2004-0408) Non-randomized ORR: 69%, CR: 62%

Chemotherapy

  • Alemtuzumab (Campath) as follows:
    • First week: 3 mg IV once on day 1, then 10 mg IV once on day 2, then 30 mg IV once on day 3
    • Subsequent weeks: 30 mg IV three times weekly
  • 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

  1. 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

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Regimen

Study Evidence
Herbaux et al. 2014 Retrospective

Chemotherapy

21-day cycle for 6 cycles

References

  1. 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

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Regimen

Study Evidence Efficacy
Ravandi et al. 2009 (MDACC 2004-0408) Non-randomized ORR: 69%, CR: 62%

Chemotherapy

  • Alemtuzumab (Campath) as follows:
    • First week: 3 mg IV once on day 1, then 10 mg IV once on day 2, then 30 mg IV once on day 3
    • Subsequent weeks: 30 mg IV three times weekly
  • 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

  1. 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

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Regimen

Study Evidence
Kornauth et al. 2019 Non-randomized

Chemotherapy

Continued indefinitely

References

  1. 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

  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 link to PMC article PubMed
  3. 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