Difference between revisions of "Staging page"

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=Guidelines=
 
=Guidelines=
 
=="How I Treat"==
 
=="How I Treat"==
−
*'''2011:''' Lamy T, Loughran TP Jr. How I treat LGL leukemia. Blood. 2011 Mar 10;117(10):2764-74. [http://www.bloodjournal.org/content/117/10/2764.long link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3062292/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/21190991 PubMed]
+
*'''2012:''' Dearden [https://ashpublications.org/blood/article/120/3/538/30480/How-I-treat-prolymphocytic-leukemia How I treat prolymphocytic leukemia]
 +
 
==[https://www.nccn.org/ NCCN]==
 
==[https://www.nccn.org/ NCCN]==
−
*[https://www.nccn.org/professionals/physician_gls/pdf/t-cell.pdf NCCN Guidelines - T-cell Lymphomas] - contains information about T-cell Large Granular Lymphocytic Leukemia
+
*[https://www.nccn.org/professionals/physician_gls/pdf/t-cell.pdf NCCN Guidelines - T-cell Lymphomas]
−
=Initial therapy=
+
=Diagnosis, staging and treatment response criteria (TPLL-ISG)=
−
==Cyclophosphamide monotherapy {{#subobject:464f08|Regimen=1}}==  
+
*'''2019:''' Staber et al. [https://doi.org/10.1182/blood.2019000402 Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia]
 +
=Upfront induction therapy=
 +
==Alemtuzumab monotherapy {{#subobject:ab5318|Regimen=1}}==
 
<div class="toccolours" style="background-color:#eeeeee">
 
<div class="toccolours" style="background-color:#eeeeee">
−
===Regimen {{#subobject:78c71b|Variant=1}}===
+
===Regimen {{#subobject:893a|Variant=1}}===
−
{| class="wikitable" style="width: 40%; text-align:center;"  
+
{| class="wikitable" style="width: 60%; text-align:center;"  
−
!style="width: 25%"|Study
+
!style="width: 33%"|Study
−
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]]
+
!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
 +
!style="width: 33%"|[[Levels_of_Evidence#Efficacy|Efficacy]]
 +
|-
 +
|[https://doi.org/10.1182/blood.v98.6.1721 Dearden et al. 2001]
 +
| style="background-color:#91cf61" |Non-randomized
 +
|ORR: 76%, CR: 60%
 
|-
 
|-
−
|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2930955/ Bareau et al. 2010]
+
|[https://doi.org/10.1182/blood-2011-08-372854 Dearden et al. 2011]
−
|style="background-color:#ffffbe"|Retrospective
+
| style="background-color:#91cf61" |Non-randomized
 +
|ORR: 91%, CR: 81%
 
|-
 
|-
 
|}
 
|}
−
''These are retrospective series, of which Bareau et al. 2010 is representative. The most common dose reported is 100 mg PO once per day; some authors mentions co-administration of steroids but details are not supplied.''
+
<div class="toccolours" style="background-color:#b3e2cd">
−
====Immunosuppressive therapy====
+
====Targeted therapy====
−
*[[Cyclophosphamide (Cytoxan)]] 50 to 100 mg PO once per day
+
* [[Alemtuzumab (Campath)]] as follows:
−
'''Lamy et al. 2011 recommends that patients who are responding are continued on treatment for 6 to 12 months; patients who do not respond within 4 months of therapy are discontinued'''
+
**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'''
 
</div></div>
 
</div></div>
 
===References===
 
===References===
−
# '''Retrospective:''' Go RS, Li CY, Tefferi A, Phyliky RL. Acquired pure red cell aplasia associated with lymphoproliferative disease of granular T lymphocytes. Blood. 2001 Jul 15;98(2):483-5. [http://www.bloodjournal.org/content/98/2/483.long link to original article] [https://pubmed.ncbi.nlm.nih.gov?term=11435321 PubMed]
+
#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. [https://doi.org/10.1182/blood.v98.6.1721 link to original article] [https://pubmed.ncbi.nlm.nih.gov/11535503 PubMed]
−
# '''Retrospective:''' Bareau B, Rey J, Hamidou M, Donadieu J, Morcet J, Reman O, Schleinitz N, Tournilhac O, Roussel M, Fest T, Lamy T. Analysis of a French cohort of patients with large granular lymphocyte leukemia: a report on 229 cases. Haematologica. 2010 Sep;95(9):1534-41. [http://www.haematologica.org/content/95/9/1534.long link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2930955/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/20378561 PubMed] content property of [http://hemonc.org HemOnc.org]
+
#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. [https://doi.org/10.1182/blood-2011-08-372854 link to original article] [https://pubmed.ncbi.nlm.nih.gov/21948296 PubMed]
−
# '''Retrospective:''' Moignet A, Hasanali Z, Zambello R, Pavan L, Bareau B, Tournilhac O, Roussel M, Fest T, Awwad A, Baab K, Semenzato G, Houot R, Loughran TP Jr, Lamy T. Cyclophosphamide as a first-line therapy in LGL leukemia. Leukemia. 2014 May;28(5):1134-6. Epub 2013 Nov 27. [https://doi.org/10.1038/leu.2013.359 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4017255/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/24280867 PubMed]
+
==Pentostatin & Alemtuzumab {{#subobject:aacb018|Regimen=1}}==
−
==Methotrexate & Prednisone {{#subobject:9c0e17|Regimen=1}}==
 
 
<div class="toccolours" style="background-color:#eeeeee">
 
<div class="toccolours" style="background-color:#eeeeee">
−
===Regimen {{#subobject:3bbb5|Variant=1}}===
+
===Regimen {{#subobject:881cj3a|Variant=1}}===
 +
{| class="wikitable" style="width: 60%; text-align:center;"
 +
!style="width: 33%"|Study
 +
!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
 +
!style="width: 33%"|[[Levels_of_Evidence#Efficacy|Efficacy]]
 +
|-
 +
|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4881363/ Ravandi et al. 2009 (MDACC 2004-0408)]
 +
| style="background-color:#91cf61" |Non-randomized
 +
|ORR: 69%, CR: 62%
 +
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 +
====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/m<sup>2</sup> IV once per week
 +
**Week 5 onwards: 4 mg/m<sup>2</sup> 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)'''
 +
</div></div>
 +
===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. [https://doi.org/10.1200/JCO.2009.22.6688 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4881363/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/19805674 PubMed]
 +
=Relapsed or refractory, salvage therapy=
 +
==Bendamustine monotherapy {{#subobject:a1kc88|Regimen=1}}==
 +
<div class="toccolours" style="background-color:#eeeeee">
 +
===Regimen {{#subobject:90ucj3a|Variant=1}}===
 
{| class="wikitable" style="width: 40%; text-align:center;"  
 
{| class="wikitable" style="width: 40%; text-align:center;"  
−
!style="width: 25%"|Study
+
! style="width: 25%" |Study
−
!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]]
+
! style="width: 25%" |[[Levels_of_Evidence#Evidence|Evidence]]
−
|-
 
−
|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4377298/ Loughran et al. 2014 (ECOG E5998)]
 
−
|style="background-color:#91cf61"|Phase 2
 
 
|-
 
|-
 +
|[https://doi.org/full/10.1111/bjh.13175 Herbaux et al. 2014]
 +
| style="background-color:#ffffbe" |Retrospective
 
|}
 
|}
 
<div class="toccolours" style="background-color:#b3e2cd">
 
<div class="toccolours" style="background-color:#b3e2cd">
−
====Immunosuppressive therapy====
+
====Chemotherapy====
−
*[[Methotrexate (MTX)]] 10 mg/m<sup>2</sup>/day PO in divided doses once per day on days 1, 8, 15, 22
+
* [[Bendamustine]] 70 to 120 mg/m<sup>2</sup> IV once per day on days 1 & 2  
−
**Details not described in ECOG E5998; Lamy et al. 2011 divides the 10 mg/m<sup>2</sup> methotrexate dose into 5 mg/m<sup>2</sup> in the morning and 5 mg/m<sup>2</sup> in the evening
+
'''21-day cycle for 6 cycles'''
−
*[[Prednisone (Sterapred)]] 1 mg/kg PO once per day for 30 days, then tapered off over 24 days (details not described)
 
−
'''28-day cycles'''
 
−
</div>
 
−
<div class="toccolours" style="background-color:#cbd5e7">
 
−
====Subsequent treatment====
 
−
*Patients with PR: continue up to one year
 
−
*Patients with CR: continue for one month past documented CR
 
−
*Non-responders were transitioned to [[#Cyclophosphamide_.26_Prednisone|cyclophosphamide & prednisone]]
 
 
</div></div>
 
</div></div>
 
===References===
 
===References===
−
# '''ECOG E5998:''' Loughran TP Jr, Zickl L, Olson TL, Wang V, Zhang D, Rajala HL, Hasanali Z, Bennett JM, Lazarus HM, Litzow MR, Evens AM, Mustjoki S, Tallman MS. Immunosuppressive therapy of LGL leukemia: prospective multicenter phase II study by the Eastern Cooperative Oncology Group (E5998). Leukemia. 2015 Apr;29(4):886-94. Epub 2014 Sep 13. [https://doi.org/10.1038/leu.2014.298 link to original article]  '''contains dosing details in manuscript''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4377298/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/25306898 PubMed]
+
# '''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. [https://doi.org/full/10.1111/bjh.13175 link to original article] [https://pubmed.ncbi.nlm.nih.gov/25316212 PubMed]
−
=Relapsed or refractory=
+
==Pentostatin & Alemtuzumab {{#subobject:a1kb018|Regimen=1}}==
−
==Cyclophosphamide & Prednisone {{#subobject:3cfd6b|Regimen=1}}==
 
 
<div class="toccolours" style="background-color:#eeeeee">
 
<div class="toccolours" style="background-color:#eeeeee">
−
===Regimen {{#subobject:c27546|Variant=1}}===
+
===Regimen {{#subobject:991cj3a|Variant=1}}===
 
{| class="wikitable" style="width: 60%; text-align:center;"  
 
{| class="wikitable" style="width: 60%; text-align:center;"  
 
!style="width: 33%"|Study
 
!style="width: 33%"|Study
Line 71: Line 97:
 
!style="width: 33%"|[[Levels_of_Evidence#Efficacy|Efficacy]]
 
!style="width: 33%"|[[Levels_of_Evidence#Efficacy|Efficacy]]
 
|-
 
|-
−
|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4377298/ Loughran et al. 2014 (ECOG E5998)]
+
|[https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4881363/ Ravandi et al. 2009 (MDACC 2004-0408)]
−
|style="background-color:#91cf61"|Phase 2
+
| style="background-color:#91cf61" |Non-randomized
−
|ORR: 64% (95% CI, 35-87)
+
|ORR: 69%, CR: 62%
 +
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 +
====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/m<sup>2</sup> IV once per week
 +
**Week 5 onwards: 4 mg/m<sup>2</sup> 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)'''
 +
</div></div>
 +
===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. [https://doi.org/10.1200/JCO.2009.22.6688 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4881363/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/19805674 PubMed]
 +
==Ibrutinib & Venetoclax {{#subobject:a1uh18|Regimen=1}}==
 +
<div class="toccolours" style="background-color:#eeeeee">
 +
===Regimen {{#subobject:991ica|Variant=1}}===
 +
{| class="wikitable" style="width: 40%; text-align:center;"
 +
!style="width: 50%"|Study
 +
!style="width: 50%"|[[Levels_of_Evidence#Evidence|Evidence]]
 +
|-
 +
|[https://ashpublications.org/blood/article/134/Supplement_1/3965/424160/Combination-of-Venetoclax-and-Ibrutinib-Increases Kornauth et al. 2019]
 +
| style="background-color:#91cf61" |Non-randomized
 
|-
 
|-
 
|}
 
|}
−
''Note: Patients with PR continue up to one year; patients with CR continue for one month past documented CR.''
 
−
<div class="toccolours" style="background-color:#cbd5e8">
 
−
====Preceding treatment====
 
−
*[[#Methotrexate_.26_Prednisone|Methotrexate & prednisone]], with treatment failure
 
−
</div>
 
 
<div class="toccolours" style="background-color:#b3e2cd">
 
<div class="toccolours" style="background-color:#b3e2cd">
−
====Immunosuppressive therapy====
+
====Targeted therapy====
−
*[[Cyclophosphamide (Cytoxan)]] 100 mg PO once per day
+
* [[Ibrutinib (Imbruvica)|Ibrutinib]] 420 mg PO once per day
−
*[[Prednisone (Sterapred)]] 1 mg/kg PO once per day for 30 days, then tapered off over 24 days (details not described)
+
* [[Venetoclax (Venclexta)|Venetoclax]] 400 to 600 mg PO once per day
−
'''28-day cycles (see note)'''
+
'''Continued indefinitely'''
 +
</div></div>
 +
===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. [https://ashpublications.org/blood/article/134/Supplement_1/3965/424160/Combination-of-Venetoclax-and-Ibrutinib-Increases link to abstract]
 +
=Consolidation therapy after upfront or salvage therapy=
 +
'''[[Allogeneic HSCT]]''' evaluation suggested in eligible patients.
 
</div></div>
 
</div></div>
 
===References===
 
===References===
−
# '''ECOG E5998:''' Loughran TP Jr, Zickl L, Olson TL, Wang V, Zhang D, Rajala HL, Hasanali Z, Bennett JM, Lazarus HM, Litzow MR, Evens AM, Mustjoki S, Tallman MS. Immunosuppressive therapy of LGL leukemia: prospective multicenter phase II study by the Eastern Cooperative Oncology Group (E5998). Leukemia. 2015 Apr;29(4):886-94. Epub 2014 Sep 13. [https://doi.org/10.1038/leu.2014.298 link to original article] '''contains dosing details in manuscript''' [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4377298/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/25306898 PubMed]
+
# 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. [https://doi.org/full/10.1111/j.1365-2141.2010.08134.x link to original article] [https://pubmed.ncbi.nlm.nih.gov/20201944 PubMed]
−
[[Category:Large granular lymphocytic leukemia regimens]]
+
# 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. [https://doi.org/10.1016/j.bbmt.2009.11.021 link to original article] [https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2839005/ link to PMC article] [https://pubmed.ncbi.nlm.nih.gov/19961946 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. [https://www.nature.com/articles/leu2011304 link to original article] [https://pubmed.ncbi.nlm.nih.gov/22116553 PubMed]
 +
[[Category:T-cell prolymphocytic leukemia regimens]]
 
[[Category:Disease-specific pages]]
 
[[Category:Disease-specific pages]]
−
[[Category:T-cell lymphomas]]
+
[[Category:T-cell leukemias]]

Revision as of 12:43, 29 October 2022

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Section editor transclusions

0 regimens on this page
0 variants on this page


Guidelines

"How I Treat"

NCCN

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

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

  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

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

  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

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

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

  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

Regimen

Study Evidence
Kornauth et al. 2019 Non-randomized

Targeted therapy

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
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