Case report: A case of severe capecitabine toxicity due to confirmed in trans compound heterozygosity of a common and rare DPYD variant

Variations in the activity of the enzyme dihydropyrimidine dehydrogenase (DPD) are associated with toxicity to fluoropyrimidine-containing chemotherapy. Testing of DPD deficiency either by targeted genotyping of the corresponding DPYD gene or by quantification of plasma concentration of uracil and d...

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Main Authors: Amy de Haar-Holleman, Pieter-Jan Cortoos, Jelle Vlaeminck, Paulien Van Landuyt, Stephane Steurbaut, Freya Vaeyens, Vincent Haufroid
Format: Article
Language:English
Published: Frontiers Media S.A. 2024-09-01
Series:Frontiers in Pharmacology
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Online Access:https://www.frontiersin.org/articles/10.3389/fphar.2024.1459565/full
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author Amy de Haar-Holleman
Amy de Haar-Holleman
Pieter-Jan Cortoos
Pieter-Jan Cortoos
Jelle Vlaeminck
Paulien Van Landuyt
Stephane Steurbaut
Stephane Steurbaut
Freya Vaeyens
Vincent Haufroid
Vincent Haufroid
author_facet Amy de Haar-Holleman
Amy de Haar-Holleman
Pieter-Jan Cortoos
Pieter-Jan Cortoos
Jelle Vlaeminck
Paulien Van Landuyt
Stephane Steurbaut
Stephane Steurbaut
Freya Vaeyens
Vincent Haufroid
Vincent Haufroid
author_sort Amy de Haar-Holleman
collection DOAJ
description Variations in the activity of the enzyme dihydropyrimidine dehydrogenase (DPD) are associated with toxicity to fluoropyrimidine-containing chemotherapy. Testing of DPD deficiency either by targeted genotyping of the corresponding DPYD gene or by quantification of plasma concentration of uracil and dihydrouracil (phenotyping approach) are the two main methods capable of predicting reduced enzymatic activity in order to reduce adverse reactions after fluoropyrimidine treatment. In this paper, we describe a patient with locally advanced colon carcinoma with severe toxicity following capecitabine therapy. Whereas targeted genotyping for the 4 most common DPYD variants analysis revealed heterozygous presence of the c.2846A>T variant, which is a relatively common variant associated with a partial deficiency, additional phenotyping was compatible with a complete DPD deficiency. Subsequent sequencing of the whole DPYD gene revealed the additional presence of the rare c.2872A>G variant, which is associated with a total loss of DPD activity. A clinical case of in trans compound heterozygosity of a common and a rare DPYD variant (c.2846A>T and c.2872A>G) has, to the best of our knowledge, not been previously described. Our case report shows the importance of performing either preemptive phenotyping or preemptive complete genetic analysis of the DPYD gene for patients planned for systemic fluoropyrimidines to identify rare and low frequency variants responsible for potentially life-threatening toxic reactions.
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spelling doaj-art-7e18b3da531047aab7807db6f624bd1d2025-08-20T02:17:25ZengFrontiers Media S.A.Frontiers in Pharmacology1663-98122024-09-011510.3389/fphar.2024.14595651459565Case report: A case of severe capecitabine toxicity due to confirmed in trans compound heterozygosity of a common and rare DPYD variantAmy de Haar-Holleman0Amy de Haar-Holleman1Pieter-Jan Cortoos2Pieter-Jan Cortoos3Jelle Vlaeminck4Paulien Van Landuyt5Stephane Steurbaut6Stephane Steurbaut7Freya Vaeyens8Vincent Haufroid9Vincent Haufroid10Department of Medical Oncology, Universitair Ziekenhuis Brussel (UZBrussel), Brussels, BelgiumTranslational Oncology Research Center (TORC), Vrije Universiteit Brussel (VUB), Brussels, BelgiumPharmacy Department, Universitair Ziekenhuis Brussel (UZBrussel), Brussels, BelgiumFaculty of Medicine & Pharmacy, Vrije Universiteit Brussel (VUB), Brussels, BelgiumCentre for Medical Genetics, Research Group Genetics, Reproduction and Development, Clinical Sciences, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel), Brussels, BelgiumPharmacy Department, Universitair Ziekenhuis Brussel (UZBrussel), Brussels, BelgiumPharmacy Department, Universitair Ziekenhuis Brussel (UZBrussel), Brussels, BelgiumFaculty of Medicine & Pharmacy, Vrije Universiteit Brussel (VUB), Brussels, BelgiumCentre for Medical Genetics, Research Group Genetics, Reproduction and Development, Clinical Sciences, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel), Brussels, BelgiumLouvain Center for Toxicology and Applied Pharmacology, Institut de Recherche Expérimentale et Clinique (IREC), Université Catholique de Louvain (UCLouvain), Brussels, BelgiumDepartment of Clinical Chemistry, Cliniques Universitaires Saint-Luc, Brussels, BelgiumVariations in the activity of the enzyme dihydropyrimidine dehydrogenase (DPD) are associated with toxicity to fluoropyrimidine-containing chemotherapy. Testing of DPD deficiency either by targeted genotyping of the corresponding DPYD gene or by quantification of plasma concentration of uracil and dihydrouracil (phenotyping approach) are the two main methods capable of predicting reduced enzymatic activity in order to reduce adverse reactions after fluoropyrimidine treatment. In this paper, we describe a patient with locally advanced colon carcinoma with severe toxicity following capecitabine therapy. Whereas targeted genotyping for the 4 most common DPYD variants analysis revealed heterozygous presence of the c.2846A>T variant, which is a relatively common variant associated with a partial deficiency, additional phenotyping was compatible with a complete DPD deficiency. Subsequent sequencing of the whole DPYD gene revealed the additional presence of the rare c.2872A>G variant, which is associated with a total loss of DPD activity. A clinical case of in trans compound heterozygosity of a common and a rare DPYD variant (c.2846A>T and c.2872A>G) has, to the best of our knowledge, not been previously described. Our case report shows the importance of performing either preemptive phenotyping or preemptive complete genetic analysis of the DPYD gene for patients planned for systemic fluoropyrimidines to identify rare and low frequency variants responsible for potentially life-threatening toxic reactions.https://www.frontiersin.org/articles/10.3389/fphar.2024.1459565/fullcase reportcapecitabineDPYD gene polymorphismpheno- and genotypingrare variant
spellingShingle Amy de Haar-Holleman
Amy de Haar-Holleman
Pieter-Jan Cortoos
Pieter-Jan Cortoos
Jelle Vlaeminck
Paulien Van Landuyt
Stephane Steurbaut
Stephane Steurbaut
Freya Vaeyens
Vincent Haufroid
Vincent Haufroid
Case report: A case of severe capecitabine toxicity due to confirmed in trans compound heterozygosity of a common and rare DPYD variant
Frontiers in Pharmacology
case report
capecitabine
DPYD gene polymorphism
pheno- and genotyping
rare variant
title Case report: A case of severe capecitabine toxicity due to confirmed in trans compound heterozygosity of a common and rare DPYD variant
title_full Case report: A case of severe capecitabine toxicity due to confirmed in trans compound heterozygosity of a common and rare DPYD variant
title_fullStr Case report: A case of severe capecitabine toxicity due to confirmed in trans compound heterozygosity of a common and rare DPYD variant
title_full_unstemmed Case report: A case of severe capecitabine toxicity due to confirmed in trans compound heterozygosity of a common and rare DPYD variant
title_short Case report: A case of severe capecitabine toxicity due to confirmed in trans compound heterozygosity of a common and rare DPYD variant
title_sort case report a case of severe capecitabine toxicity due to confirmed in trans compound heterozygosity of a common and rare dpyd variant
topic case report
capecitabine
DPYD gene polymorphism
pheno- and genotyping
rare variant
url https://www.frontiersin.org/articles/10.3389/fphar.2024.1459565/full
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