SYSTEMIC MASTOCYTOSIS: MOLECULAR LANDSCAPE AND IMPLICATIONS FOR TREATMENT

Over the past decade we have witnessed major advances in the molecular characterization of systemic mastocytosis (SM). This has provided important information for a better understanding of the pathogenesis of the disease but has also practically impacted on the way we diagnose and manage it. Advance...

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Main Authors: Cecilia Monaldi, Sara De Santis, Manuela Mancini, Samantha Bruno, Michele Cavo, Simona Soverini
Format: Article
Language:English
Published: PAGEPress Publications 2021-06-01
Series:Mediterranean Journal of Hematology and Infectious Diseases
Online Access:https://www.mjhid.org/index.php/mjhid/article/view/4666
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author Cecilia Monaldi
Sara De Santis
Manuela Mancini
Samantha Bruno
Michele Cavo
Simona Soverini
author_facet Cecilia Monaldi
Sara De Santis
Manuela Mancini
Samantha Bruno
Michele Cavo
Simona Soverini
author_sort Cecilia Monaldi
collection DOAJ
description Over the past decade we have witnessed major advances in the molecular characterization of systemic mastocytosis (SM). This has provided important information for a better understanding of the pathogenesis of the disease but has also practically impacted on the way we diagnose and manage it. Advances in molecular testing has indeed run in parallel with advances in therapeutic targeting of constitutive active KIT, the major driver of the disease. Assessing the molecular landscape in each individual SM patient is nowadays important for diagnosis, prognosis, treatment and monitoring of therapeutic efficacy. This is facilitated by the routine availability of novel technologies like digital PCR and NGS. This review aims to summarize the pathogenesis of the disease, discuss the value of molecular diagnostic testing and how it should be performed, and provide an overview of present and future therapeutic concepts based on fine molecular characterization of SM patients.
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institution Kabale University
issn 2035-3006
language English
publishDate 2021-06-01
publisher PAGEPress Publications
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series Mediterranean Journal of Hematology and Infectious Diseases
spelling doaj-art-052cdf27751e46f1aacf57ad9e044d1f2025-01-02T00:23:24ZengPAGEPress PublicationsMediterranean Journal of Hematology and Infectious Diseases2035-30062021-06-0113110.4084/MJHID.2021.046SYSTEMIC MASTOCYTOSIS: MOLECULAR LANDSCAPE AND IMPLICATIONS FOR TREATMENTCecilia Monaldi0Sara De SantisManuela ManciniSamantha BrunoMichele CavoSimona Soverinia:1:{s:5:"en_US";s:106:"Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Università di Bologna, Bologna, Italy";}Over the past decade we have witnessed major advances in the molecular characterization of systemic mastocytosis (SM). This has provided important information for a better understanding of the pathogenesis of the disease but has also practically impacted on the way we diagnose and manage it. Advances in molecular testing has indeed run in parallel with advances in therapeutic targeting of constitutive active KIT, the major driver of the disease. Assessing the molecular landscape in each individual SM patient is nowadays important for diagnosis, prognosis, treatment and monitoring of therapeutic efficacy. This is facilitated by the routine availability of novel technologies like digital PCR and NGS. This review aims to summarize the pathogenesis of the disease, discuss the value of molecular diagnostic testing and how it should be performed, and provide an overview of present and future therapeutic concepts based on fine molecular characterization of SM patients.https://www.mjhid.org/index.php/mjhid/article/view/4666
spellingShingle Cecilia Monaldi
Sara De Santis
Manuela Mancini
Samantha Bruno
Michele Cavo
Simona Soverini
SYSTEMIC MASTOCYTOSIS: MOLECULAR LANDSCAPE AND IMPLICATIONS FOR TREATMENT
Mediterranean Journal of Hematology and Infectious Diseases
title SYSTEMIC MASTOCYTOSIS: MOLECULAR LANDSCAPE AND IMPLICATIONS FOR TREATMENT
title_full SYSTEMIC MASTOCYTOSIS: MOLECULAR LANDSCAPE AND IMPLICATIONS FOR TREATMENT
title_fullStr SYSTEMIC MASTOCYTOSIS: MOLECULAR LANDSCAPE AND IMPLICATIONS FOR TREATMENT
title_full_unstemmed SYSTEMIC MASTOCYTOSIS: MOLECULAR LANDSCAPE AND IMPLICATIONS FOR TREATMENT
title_short SYSTEMIC MASTOCYTOSIS: MOLECULAR LANDSCAPE AND IMPLICATIONS FOR TREATMENT
title_sort systemic mastocytosis molecular landscape and implications for treatment
url https://www.mjhid.org/index.php/mjhid/article/view/4666
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AT samanthabruno systemicmastocytosismolecularlandscapeandimplicationsfortreatment
AT michelecavo systemicmastocytosismolecularlandscapeandimplicationsfortreatment
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