Sustained MRD negative remission in del17p and TP53 mutated B cell prolymphocytic leukemia with ibrutinib and venetoclax

B cell prolymphocytic leukemia is a rare and aggressive disorder often with high risk features including TP53 mutation, deletion 17p and complex karyotype. There is scarcity of data regarding treatment and existing therapies induce short lived remissions. Ibrutinib, a Bruton tyrosine kinase inhibito...

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Main Authors: Maria Tariq Siddiqui, Allyson Price, Alessandra Ferrajoli, Gautam Borthakur
Format: Article
Language:English
Published: Elsevier 2021-01-01
Series:Leukemia Research Reports
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S2213048921000339
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author Maria Tariq Siddiqui
Allyson Price
Alessandra Ferrajoli
Gautam Borthakur
author_facet Maria Tariq Siddiqui
Allyson Price
Alessandra Ferrajoli
Gautam Borthakur
author_sort Maria Tariq Siddiqui
collection DOAJ
description B cell prolymphocytic leukemia is a rare and aggressive disorder often with high risk features including TP53 mutation, deletion 17p and complex karyotype. There is scarcity of data regarding treatment and existing therapies induce short lived remissions. Ibrutinib, a Bruton tyrosine kinase inhibitor, has had success in some patients with high risk features. Venetoclax, a BCL-2 inhibitor, has primarily been used in the relapsed setting. We present a case of B PLL with deletion 17p and mutated TP53 treated with ibrutinib and venetoclax in the frontline setting which resulted in measurable/minimal residual disease negative remission for approximately three years.
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spelling doaj.art-2d8fc129dfff4383b6953837cca4d2de2022-12-21T18:37:10ZengElsevierLeukemia Research Reports2213-04892021-01-0116100266Sustained MRD negative remission in del17p and TP53 mutated B cell prolymphocytic leukemia with ibrutinib and venetoclaxMaria Tariq Siddiqui0Allyson Price1Alessandra Ferrajoli2Gautam Borthakur3Corresponding author.; The Department of Leukemia, MD Anderson Cancer Center, Houston, TX, United StatesThe Department of Leukemia, MD Anderson Cancer Center, Houston, TX, United StatesThe Department of Leukemia, MD Anderson Cancer Center, Houston, TX, United StatesThe Department of Leukemia, MD Anderson Cancer Center, Houston, TX, United StatesB cell prolymphocytic leukemia is a rare and aggressive disorder often with high risk features including TP53 mutation, deletion 17p and complex karyotype. There is scarcity of data regarding treatment and existing therapies induce short lived remissions. Ibrutinib, a Bruton tyrosine kinase inhibitor, has had success in some patients with high risk features. Venetoclax, a BCL-2 inhibitor, has primarily been used in the relapsed setting. We present a case of B PLL with deletion 17p and mutated TP53 treated with ibrutinib and venetoclax in the frontline setting which resulted in measurable/minimal residual disease negative remission for approximately three years.http://www.sciencedirect.com/science/article/pii/S2213048921000339B cell prolymphocytic leukemiaIbrutinibVenetoclaxDeletion 17pTP53
spellingShingle Maria Tariq Siddiqui
Allyson Price
Alessandra Ferrajoli
Gautam Borthakur
Sustained MRD negative remission in del17p and TP53 mutated B cell prolymphocytic leukemia with ibrutinib and venetoclax
Leukemia Research Reports
B cell prolymphocytic leukemia
Ibrutinib
Venetoclax
Deletion 17p
TP53
title Sustained MRD negative remission in del17p and TP53 mutated B cell prolymphocytic leukemia with ibrutinib and venetoclax
title_full Sustained MRD negative remission in del17p and TP53 mutated B cell prolymphocytic leukemia with ibrutinib and venetoclax
title_fullStr Sustained MRD negative remission in del17p and TP53 mutated B cell prolymphocytic leukemia with ibrutinib and venetoclax
title_full_unstemmed Sustained MRD negative remission in del17p and TP53 mutated B cell prolymphocytic leukemia with ibrutinib and venetoclax
title_short Sustained MRD negative remission in del17p and TP53 mutated B cell prolymphocytic leukemia with ibrutinib and venetoclax
title_sort sustained mrd negative remission in del17p and tp53 mutated b cell prolymphocytic leukemia with ibrutinib and venetoclax
topic B cell prolymphocytic leukemia
Ibrutinib
Venetoclax
Deletion 17p
TP53
url http://www.sciencedirect.com/science/article/pii/S2213048921000339
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