CHILDHOOD MASTOCYTOSIS NEW DEVELOPMENTS IN DIAGNOSIS AND TREATMENT

CHI775LDHOOD_MASTOCYTOSI775S_NEW_DEVELOPMENTS_IN_DI775AGNOSI775S_AND_TREATMENTKAPAK

PATHOGENESIS OF CHILDHOOD MASTOCYTOSIS

Zuhal Karalı1
Yasin Karalı2

1Bursa City Hospital, Department of Pediatric Immunology and Allergy, Bursa, Türkiye
2Bursa Uludağ University, Faculty of Medicine, Department of Pediatric Immunology, Bursa, Türkiye

Karalı Z, Karalı Y. Pathogenesis of Childhood Mastocytosis. In: Özdemir Ö, editor. Childhood Mastocytosis: New Developments in Diagnosis and Treatment. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.21-34.

ABSTRACT

Mast cells are involved in IgE-mediated immediate allergic responses. Mastocytosis occurs due to a dense accumulation of mast cells in tissues and the mediators they release. At the center of this process is stem cell factor (SCF), a vital growth factor that binds to the c-KIT receptor, a transmembrane recep- tor associated with tyrosine kinases on mast cells. The KIT receptor (CD117) is classified as a type III receptor tyrosine kinase. The interaction of SCF and KIT regulates a critical process controlling mast cell exchange, movement, accumulation, and survival in tissues. Defects in this interaction impair mast cell growth, differentiation, apoptosis, and activation, leading to the development of mastocytosis. Other factors involved in the pathogenesis of mastocytosis include three groups of mast cell mediators: synthesized stored mediators, newly synthesized lipid mediators, and cytokines/chemokines.

Keywords: Mastocytosis; c-KIT mutation; Stem cell factor; Mast cell mediators

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