Difference between revisions of "T-cell prolymphocytic leukemia"

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! colspan="2" align="center" style="color:white; font-size:125%; background-color:#de2d26" |'''Section editor'''
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[[#top|Back to Top]]
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| style="background-color:#F0F0F0" |[[File:Bdholaria.jpg|frameless|upright=0.3|center]]
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</div>
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|<big>[[User:Bdholaria|Bhagirathbhai Dholaria, MBBS]]<br>Vanderbilt University<br>Nashville, TN</big>
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{{#lst:Editorial board transclusions|tcl}}
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*''We have moved [[How I Treat]] articles to a dedicated page.''
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{{TOC limit|limit=3}}
 
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=Guidelines=
 
=Guidelines=
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==[https://www.nccn.org/ NCCN]==
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'''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.'''
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==NCCN==
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*[https://www.nccn.org/professionals/physician_gls/pdf/t-cell.pdf NCCN Guidelines - T-cell Lymphomas]
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*''NCCN does not currently have guidelines at this granular level; please see [https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1483 NCCN Guidelines - T-cell Lymphomas].''
  
 
=Diagnosis, staging and treatment response criteria (TPLL-ISG)=
 
=Diagnosis, staging and treatment response criteria (TPLL-ISG)=
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*'''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]
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*'''2019:''' Staber et al. [https://ashpublications.org/blood/article-lookup/doi/10.1182/blood.2019000402 Consensus criteria for diagnosis, staging, and treatment response assessment of T-cell prolymphocytic leukemia]
 
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=Upfront induction therapy=
 
=Upfront induction therapy=
 
==Alemtuzumab monotherapy {{#subobject:ab5318|Regimen=1}}==
 
==Alemtuzumab monotherapy {{#subobject:ab5318|Regimen=1}}==
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<div class="toccolours" style="background-color:#eeeeee">
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|-
 
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|[[#top|back to top]]
 
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|}
 
 
===Regimen {{#subobject:893a|Variant=1}}===
 
===Regimen {{#subobject:893a|Variant=1}}===
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{| class="wikitable" style="width: 75%; text-align:center;"  
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{| class="wikitable sortable" style="width: 80%; text-align:center;"  
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!style="width: 33%"|Study
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!style="width: 25%"|Study
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!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
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!style="width: 25%"|Dates of enrollment
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!style="width: 33%"|[[Levels_of_Evidence#Efficacy|Efficacy]]
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!style="width: 25%"|[[Levels_of_Evidence#Evidence|Evidence]]
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!style="width: 25%"|[[Levels_of_Evidence#Efficacy|Efficacy]]
 
|-
 
|-
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|[https://ashpublications.org/blood/article-lookup/doi/10.1182/blood.v98.6.1721 Dearden et al. 2001]
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|[https://doi.org/10.1182/blood.v98.6.1721 Dearden et al. 2001]
 +
|1993-03 to 2000-05
 
| style="background-color:#91cf61" |Non-randomized
 
| style="background-color:#91cf61" |Non-randomized
 
|ORR: 76%, CR: 60%
 
|ORR: 76%, CR: 60%
 
|-
 
|-
−
|[https://ashpublications.org/blood/article-lookup/doi/10.1182/blood-2011-08-372854 Dearden et al. 2011]
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|[https://doi.org/10.1182/blood-2011-08-372854 Dearden et al. 2011 (UKCLL05)]
 +
|2005-10 to 2007-07
 
| style="background-color:#91cf61" |Non-randomized
 
| style="background-color:#91cf61" |Non-randomized
 
|ORR: 91%, CR: 81%
 
|ORR: 91%, CR: 81%
 
|-
 
|-
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Targeted therapy====
 
====Targeted therapy====
−
 
 
* [[Alemtuzumab (Campath)]] as follows:
 
* [[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
+
**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
−
**Subsequent weeks: 30 mg IV three times weekly  
+
**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'''
 
'''Continued until achievement of CR or best response or for up to a total of 3 months'''
−
 
+
</div></div>
 
===References===
 
===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. [https://doi.org/10.1182/blood.v98.6.1721 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/11535503/ 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://ashpublications.org/blood/article-lookup/doi/10.1182/blood.v98.6.1721 link to original article] [https://www.ncbi.nlm.nih.gov/pubmed/11535503 PubMed]
+
#'''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. [https://doi.org/10.1182/blood-2011-08-372854 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/21948296/ PubMed] EudraCT 2004-004636-31
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#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://ashpublications.org/blood/article-lookup/doi/10.1182/blood-2011-08-372854 link to original article] [https://www.ncbi.nlm.nih.gov/pubmed/21948296 PubMed]
 
  
 
==Pentostatin & Alemtuzumab {{#subobject:aacb018|Regimen=1}}==
 
==Pentostatin & Alemtuzumab {{#subobject:aacb018|Regimen=1}}==
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<div class="toccolours" style="background-color:#eeeeee">
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|[[#top|back to top]]
 
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===Regimen {{#subobject:881cj3a|Variant=1}}===
 
===Regimen {{#subobject:881cj3a|Variant=1}}===
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{| class="wikitable" style="width: 50%; text-align:center;"  
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{| class="wikitable" style="width: 60%; text-align:center;"  
 
!style="width: 33%"|Study
 
!style="width: 33%"|Study
 
!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
 
!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
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|ORR: 69%, CR: 62%
 
|ORR: 69%, CR: 62%
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Targeted therapy====
 
====Targeted therapy====
−
 
 
*[[Alemtuzumab (Campath)]] as follows:
 
*[[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
+
**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
−
**Subsequent weeks: 30 mg IV three times weekly  
+
**Week 2 onwards: 30 mg IV three times weekly  
 
====Chemotherapy====
 
====Chemotherapy====
 
*[[Pentostatin (Nipent)]] as follows:
 
*[[Pentostatin (Nipent)]] as follows:
 
**Weeks 1 to 4: 4 mg/m<sup>2</sup> IV once per week
 
**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
 
**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)'''
 
'''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===
 
===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://www.ncbi.nlm.nih.gov/pubmed/19805674 PubMed]
+
#'''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] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/19805674/ PubMed]
  
 
=Relapsed or refractory, salvage therapy=
 
=Relapsed or refractory, salvage therapy=
 
==Bendamustine monotherapy {{#subobject:a1kc88|Regimen=1}}==
 
==Bendamustine monotherapy {{#subobject:a1kc88|Regimen=1}}==
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<div class="toccolours" style="background-color:#eeeeee">
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|-
 
−
|[[#top|back to top]]
 
−
|}
 
 
===Regimen {{#subobject:90ucj3a|Variant=1}}===
 
===Regimen {{#subobject:90ucj3a|Variant=1}}===
−
{| class="wikitable" style="width: 50%; text-align:center;"  
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{| 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://onlinelibrary.wiley.com/doi/full/10.1111/bjh.13175 Herbaux et al. 2014]
+
|[https://doi.org/10.1111/bjh.13175 Herbaux et al. 2014]
 
| style="background-color:#ffffbe" |Retrospective
 
| style="background-color:#ffffbe" |Retrospective
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Chemotherapy====
 
====Chemotherapy====
−
* [[Bendamustine]] 70 to 120 mg/m<sup>2</sup> IV once per day on days 1 & 2  
+
* [[Bendamustine]] 70 to 120 mg/m<sup>2</sup> IV over 30 to 60 minutes once per day on days 1 & 2  
−
 
 
 
'''21-day cycle for 6 cycles'''
 
'''21-day cycle for 6 cycles'''
−
 
+
</div></div>
 
===References===
 
===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. [https://doi.org/10.1111/bjh.13175 link to original article] '''contains dosing details in manuscript''' [https://pubmed.ncbi.nlm.nih.gov/25316212/ 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://onlinelibrary.wiley.com/doi/full/10.1111/bjh.13175 link to original article] [https://www.ncbi.nlm.nih.gov/pubmed/25316212 PubMed]
 
  
 
==Pentostatin & Alemtuzumab {{#subobject:a1kb018|Regimen=1}}==
 
==Pentostatin & Alemtuzumab {{#subobject:a1kb018|Regimen=1}}==
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<div class="toccolours" style="background-color:#eeeeee">
−
|-
 
−
|[[#top|back to top]]
 
−
|}
 
 
===Regimen {{#subobject:991cj3a|Variant=1}}===
 
===Regimen {{#subobject:991cj3a|Variant=1}}===
−
{| class="wikitable" style="width: 50%; text-align:center;"  
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{| class="wikitable" style="width: 60%; text-align:center;"  
 
!style="width: 33%"|Study
 
!style="width: 33%"|Study
 
!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
 
!style="width: 33%"|[[Levels_of_Evidence#Evidence|Evidence]]
Line 124: Line 107:
 
|ORR: 69%, CR: 62%
 
|ORR: 69%, CR: 62%
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Targeted therapy====
 
====Targeted therapy====
−
 
 
*[[Alemtuzumab (Campath)]] as follows:
 
*[[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
+
**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
−
**Subsequent weeks: 30 mg IV three times weekly  
+
**Week 2 onwards: 30 mg IV three times weekly  
 
====Chemotherapy====
 
====Chemotherapy====
 
*[[Pentostatin (Nipent)]] as follows:
 
*[[Pentostatin (Nipent)]] as follows:
 
**Weeks 1 to 4: 4 mg/m<sup>2</sup> IV once per week
 
**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
 
**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)'''
 
'''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===
 
===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://www.ncbi.nlm.nih.gov/pubmed/19805674 PubMed]
+
#'''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}}==
 
==Ibrutinib & Venetoclax {{#subobject:a1uh18|Regimen=1}}==
−
{| class="wikitable" style="float:right; margin-left: 5px;"
+
<div class="toccolours" style="background-color:#eeeeee">
−
|-
 
−
|[[#top|back to top]]
 
−
|}
 
 
===Regimen {{#subobject:991ica|Variant=1}}===
 
===Regimen {{#subobject:991ica|Variant=1}}===
−
{| class="wikitable" style="width: 50%; text-align:center;"  
+
{| class="wikitable" style="width: 40%; text-align:center;"  
 
!style="width: 50%"|Study
 
!style="width: 50%"|Study
 
!style="width: 50%"|[[Levels_of_Evidence#Evidence|Evidence]]
 
!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]
 
|[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
+
|style="background-color:#ffffbe"|Case series
 
|-
 
|-
 
|}
 
|}
 +
<div class="toccolours" style="background-color:#b3e2cd">
 
====Targeted therapy====
 
====Targeted therapy====
 
* [[Ibrutinib (Imbruvica)|Ibrutinib]] 420 mg PO once per day
 
* [[Ibrutinib (Imbruvica)|Ibrutinib]] 420 mg PO once per day
 
* [[Venetoclax (Venclexta)|Venetoclax]] 400 to 600 mg PO once per day
 
* [[Venetoclax (Venclexta)|Venetoclax]] 400 to 600 mg PO once per day
−
 
 
'''Continued indefinitely'''
 
'''Continued indefinitely'''
−
 
+
</div></div>
 
===References===
 
===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]
 
# '''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=
 
=Consolidation therapy after upfront or salvage therapy=
−
 
 
'''[[Allogeneic HSCT]]''' evaluation suggested in eligible patients.  
 
'''[[Allogeneic HSCT]]''' evaluation suggested in eligible patients.  
−
 
+
</div></div>
 
===References===
 
===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. [https://onlinelibrary.wiley.com/doi/full/10.1111/j.1365-2141.2010.08134.x link to original article] [https://www.ncbi.nlm.nih.gov/pubmed/20201944 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/10.1111/j.1365-2141.2010.08134.x link to original article] [https://pubmed.ncbi.nlm.nih.gov/20201944/ 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. [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://www.ncbi.nlm.nih.gov/pubmed/19961946 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. [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://www.ncbi.nlm.nih.gov/pubmed/22116553 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]
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[[Category:T-cell prolymphocytic leukemia regimens]]
 
[[Category:T-cell prolymphocytic leukemia regimens]]
 
[[Category:Disease-specific pages]]
 
[[Category:Disease-specific pages]]
 
[[Category:T-cell leukemias]]
 
[[Category:T-cell leukemias]]

Revision as of 11:36, 15 May 2024

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Section editor
Bdholaria.jpg
 Bhagirathbhai Dholaria, MBBS
Vanderbilt University
Nashville, TN, USA
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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

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

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

  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 contains dosing details in manuscript PubMed
  2. 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 contains dosing details in manuscript PubMed EudraCT 2004-004636-31

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 contains dosing details in manuscript PubMed

Relapsed or refractory, salvage therapy

Bendamustine monotherapy

Regimen

Study Evidence
Herbaux et al. 2014 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

  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. Epub 2014 Oct 15. link to original article contains dosing details in manuscript 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 Case series

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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