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A Comprehensive Review of Mastocytosis From Pathophysiology to Management Strategies

Authors :
Magdalena Marchaj
Bartosz Basiaga
Klaudia Żądecka-Kobiałka
Anna Putra
Karolina Bednarz
Monika Nowicka-Jasińska
Paweł Frączyk
Sara Bieniek-Zając
Sebastian Baczkowski
Klaudia Krawiec
Source :
Journal of Education, Health and Sport, Vol 66 (2024)
Publication Year :
2024
Publisher :
Kazimierz Wielki University, 2024.

Abstract

Introduction: Mastocytosis is a rare hematologic neoplasm characterized by the abnormal proliferation and accumulation of mast cells in various tissues. Its clinical manifestations vary widely, ranging from cutaneous lesions to systemic involvement with potentially life-threatening symptoms. Understanding the pathophysiology, diagnosis, and management of mastocytosis is crucial for improving patient outcomes. Aim of the Study: The aim of this study was to provide a comprehensive overview of mastocytosis, including its epidemiology, risk factors, pathophysiology, clinical manifestations, diagnostic approaches, and management strategies. By synthesizing current knowledge on mastocytosis, this study aims to enhance understanding of the disease and guide clinical practice. Description of the State of Knowledge: Mastocytosis is classified into cutaneous and systemic forms, with various subtypes based on clinical and histopathological features. Diagnosis relies on a combination of clinical suspicion, serum tryptase levels, histological examination of bone marrow biopsies, and genetic testing. Management strategies include symptomatic treatment, avoidance of triggers, and targeted therapies such as monoclonal antibodies and tyrosine kinase inhibitors. Advanced forms may require cytoreductive therapy or allogeneic hematopoietic stem cell transplantation (alloHSCT). Conclusions: Despite advancements in diagnosis and treatment, achieving lasting remission in mastocytosis remains challenging, especially in advanced cases. Further research into the molecular mechanisms underlying the disease and the development of novel therapeutic modalities are needed to improve patient outcomes and quality of life.

Details

Language :
English, Spanish; Castilian, Polish, Russian, Ukrainian
ISSN :
23918306
Volume :
66
Database :
Directory of Open Access Journals
Journal :
Journal of Education, Health and Sport
Publication Type :
Academic Journal
Accession number :
edsdoj.f2ea492a0f9c493692ee23b2ce8fd6ef
Document Type :
article
Full Text :
https://doi.org/10.12775/JEHS.2024.66.015