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How I diagnose and manage Philadelphia chromosome-like acute lymphoblastic leukemia

Advances in our understanding of mechanisms of leukemogenesis and driver mutations in acute lymphoblastic leukemia (ALL) lead to a more precise and informative sub-classification, mainly of B-cell ALL. In parallel, in recent years, novel agents have been approved for the therapy of B-cell ALL, and m...

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Autores principales: Frisch, Avraham, Ofran, Yishai
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Ferrata Storti Foundation 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6821607/
https://www.ncbi.nlm.nih.gov/pubmed/31582548
http://dx.doi.org/10.3324/haematol.2018.207506
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author Frisch, Avraham
Ofran, Yishai
author_facet Frisch, Avraham
Ofran, Yishai
author_sort Frisch, Avraham
collection PubMed
description Advances in our understanding of mechanisms of leukemogenesis and driver mutations in acute lymphoblastic leukemia (ALL) lead to a more precise and informative sub-classification, mainly of B-cell ALL. In parallel, in recent years, novel agents have been approved for the therapy of B-cell ALL, and many others are in active clinical research. Among the newly recognized disease subtypes, Philadelphia-chromosome-like ALL is the most heterogeneous and thus, diagnostically challenging. Given that this subtype of B-cell ALL is associated with a poorer prognosis, improvement of available therapeutic approaches and protocols is a burning issue. Herein, we summarize, in a clinically relevant manner, up-to-date information regarding diagnostic strategies developed for the identification of patients with Philadelphia-chromosome-like ALL. Common therapeutic dilemmas, presented as several case scenarios, are also discussed. It is currently acceptable that patients with B-cell ALL, treated with an aim of cure, irrespective of their age, be evaluated for a Philadelphia-chromosome-like signature as early as possible. Following Philadelphia-chromosome-like recognition, a higher risk of resistance or relapse must be realized and treatment should be modified based on the patient’s specific genetic driver and clinical features. However, while active targeted therapeutic options are limited, there is much more to do than just prescribe a matched inhibitor to the identified mutated driver genes. In this review, we present a comprehensive evidence-based approach to the diagnosis and management of Philadelphia-chromosome-like ALL at different time-points during the disease course.
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spelling pubmed-68216072019-11-05 How I diagnose and manage Philadelphia chromosome-like acute lymphoblastic leukemia Frisch, Avraham Ofran, Yishai Haematologica Review Article Advances in our understanding of mechanisms of leukemogenesis and driver mutations in acute lymphoblastic leukemia (ALL) lead to a more precise and informative sub-classification, mainly of B-cell ALL. In parallel, in recent years, novel agents have been approved for the therapy of B-cell ALL, and many others are in active clinical research. Among the newly recognized disease subtypes, Philadelphia-chromosome-like ALL is the most heterogeneous and thus, diagnostically challenging. Given that this subtype of B-cell ALL is associated with a poorer prognosis, improvement of available therapeutic approaches and protocols is a burning issue. Herein, we summarize, in a clinically relevant manner, up-to-date information regarding diagnostic strategies developed for the identification of patients with Philadelphia-chromosome-like ALL. Common therapeutic dilemmas, presented as several case scenarios, are also discussed. It is currently acceptable that patients with B-cell ALL, treated with an aim of cure, irrespective of their age, be evaluated for a Philadelphia-chromosome-like signature as early as possible. Following Philadelphia-chromosome-like recognition, a higher risk of resistance or relapse must be realized and treatment should be modified based on the patient’s specific genetic driver and clinical features. However, while active targeted therapeutic options are limited, there is much more to do than just prescribe a matched inhibitor to the identified mutated driver genes. In this review, we present a comprehensive evidence-based approach to the diagnosis and management of Philadelphia-chromosome-like ALL at different time-points during the disease course. Ferrata Storti Foundation 2019-11 /pmc/articles/PMC6821607/ /pubmed/31582548 http://dx.doi.org/10.3324/haematol.2018.207506 Text en Copyright© 2019 Ferrata Storti Foundation Material published in Haematologica is covered by copyright. All rights are reserved to the Ferrata Storti Foundation. Use of published material is allowed under the following terms and conditions: https://creativecommons.org/licenses/by-nc/4.0/legalcode. Copies of published material are allowed for personal or internal use. Sharing published material for non-commercial purposes is subject to the following conditions: https://creativecommons.org/licenses/by-nc/4.0/legalcode, sect. 3. Reproducing and sharing published material for commercial purposes is not allowed without permission in writing from the publisher.
spellingShingle Review Article
Frisch, Avraham
Ofran, Yishai
How I diagnose and manage Philadelphia chromosome-like acute lymphoblastic leukemia
title How I diagnose and manage Philadelphia chromosome-like acute lymphoblastic leukemia
title_full How I diagnose and manage Philadelphia chromosome-like acute lymphoblastic leukemia
title_fullStr How I diagnose and manage Philadelphia chromosome-like acute lymphoblastic leukemia
title_full_unstemmed How I diagnose and manage Philadelphia chromosome-like acute lymphoblastic leukemia
title_short How I diagnose and manage Philadelphia chromosome-like acute lymphoblastic leukemia
title_sort how i diagnose and manage philadelphia chromosome-like acute lymphoblastic leukemia
topic Review Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6821607/
https://www.ncbi.nlm.nih.gov/pubmed/31582548
http://dx.doi.org/10.3324/haematol.2018.207506
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