TP53 in Myelodysplastic Syndromes

Myelodysplastic syndromes (MDSs) are heterogeneous for their morphology, clinical characteristics, survival of patients, and evolution to acute myeloid leukemia. Different prognostic scoring systems including the International Prognostic Scoring System (IPSS), the Revised IPSS, the WHO Typed Prognos...

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Autores principales:	Yan Jiang, Su-Jun Gao, Benoit Soubise, Nathalie Douet-Guilbert, Zi-Ling Liu, Marie-Bérengère Troadec
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	<i>TP53</i> myelodysplastic syndromes mutation monoallelic biallelic target therapy Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
Acceso en línea:	https://doaj.org/article/ec8b25c4a03740d0ac190ec97f3a4263
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spelling	oai:doaj.org-article:ec8b25c4a03740d0ac190ec97f3a42632021-11-11T15:29:47ZTP53 in Myelodysplastic Syndromes10.3390/cancers132153922072-6694https://doaj.org/article/ec8b25c4a03740d0ac190ec97f3a42632021-10-01T00:00:00Zhttps://www.mdpi.com/2072-6694/13/21/5392https://doaj.org/toc/2072-6694Myelodysplastic syndromes (MDSs) are heterogeneous for their morphology, clinical characteristics, survival of patients, and evolution to acute myeloid leukemia. Different prognostic scoring systems including the International Prognostic Scoring System (IPSS), the Revised IPSS, the WHO Typed Prognostic Scoring System, and the Lower-Risk Prognostic Scoring System have been introduced for categorizing the highly variable clinical outcomes. However, not considered by current MDS prognosis classification systems, gene variants have been identified for their contribution to the clinical heterogeneity of the disease and their impact on the prognosis. Notably, <i>TP53</i> mutation is independently associated with a higher risk category, resistance to conventional therapies, rapid transformation to leukemia, and a poor outcome. Herein, we discuss the features of monoallelic and biallelic <i>TP53</i> mutations within MDS, their corresponding carcinogenic mechanisms, their predictive value in current standard treatments including hypomethylating agents, allogeneic hematopoietic stem cell transplantation, and lenalidomide, together with the latest progress in TP53-targeted therapy strategies, especially MDS clinical trial data.Yan JiangSu-Jun GaoBenoit SoubiseNathalie Douet-GuilbertZi-Ling LiuMarie-Bérengère TroadecMDPI AGarticle<i>TP53</i>myelodysplastic syndromesmutationmonoallelicbiallelictarget therapyNeoplasms. Tumors. Oncology. Including cancer and carcinogensRC254-282ENCancers, Vol 13, Iss 5392, p 5392 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	<i>TP53</i> myelodysplastic syndromes mutation monoallelic biallelic target therapy Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282
spellingShingle	<i>TP53</i> myelodysplastic syndromes mutation monoallelic biallelic target therapy Neoplasms. Tumors. Oncology. Including cancer and carcinogens RC254-282 Yan Jiang Su-Jun Gao Benoit Soubise Nathalie Douet-Guilbert Zi-Ling Liu Marie-Bérengère Troadec TP53 in Myelodysplastic Syndromes
description	Myelodysplastic syndromes (MDSs) are heterogeneous for their morphology, clinical characteristics, survival of patients, and evolution to acute myeloid leukemia. Different prognostic scoring systems including the International Prognostic Scoring System (IPSS), the Revised IPSS, the WHO Typed Prognostic Scoring System, and the Lower-Risk Prognostic Scoring System have been introduced for categorizing the highly variable clinical outcomes. However, not considered by current MDS prognosis classification systems, gene variants have been identified for their contribution to the clinical heterogeneity of the disease and their impact on the prognosis. Notably, <i>TP53</i> mutation is independently associated with a higher risk category, resistance to conventional therapies, rapid transformation to leukemia, and a poor outcome. Herein, we discuss the features of monoallelic and biallelic <i>TP53</i> mutations within MDS, their corresponding carcinogenic mechanisms, their predictive value in current standard treatments including hypomethylating agents, allogeneic hematopoietic stem cell transplantation, and lenalidomide, together with the latest progress in TP53-targeted therapy strategies, especially MDS clinical trial data.
format	article
author	Yan Jiang Su-Jun Gao Benoit Soubise Nathalie Douet-Guilbert Zi-Ling Liu Marie-Bérengère Troadec
author_facet	Yan Jiang Su-Jun Gao Benoit Soubise Nathalie Douet-Guilbert Zi-Ling Liu Marie-Bérengère Troadec
author_sort	Yan Jiang
title	TP53 in Myelodysplastic Syndromes
title_short	TP53 in Myelodysplastic Syndromes
title_full	TP53 in Myelodysplastic Syndromes
title_fullStr	TP53 in Myelodysplastic Syndromes
title_full_unstemmed	TP53 in Myelodysplastic Syndromes
title_sort	tp53 in myelodysplastic syndromes
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/ec8b25c4a03740d0ac190ec97f3a4263
work_keys_str_mv	AT yanjiang tp53inmyelodysplasticsyndromes AT sujungao tp53inmyelodysplasticsyndromes AT benoitsoubise tp53inmyelodysplasticsyndromes AT nathaliedouetguilbert tp53inmyelodysplasticsyndromes AT zilingliu tp53inmyelodysplasticsyndromes AT marieberengeretroadec tp53inmyelodysplasticsyndromes
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TP53 in Myelodysplastic Syndromes

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