CHILDHOOD MASTOCYTOSIS NEW DEVELOPMENTS IN DIAGNOSIS AND TREATMENT

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CHILDHOOD MASTOCYTOSIS, NUTRITION AND FOOD ALLERGY

Seda Tunca1
Ayşe Aygün2

1İzmir City Hospital, Department of Pediatric Immunology and Allergy, İzmir, Türkiye
2İzmir City Hospital, Department of Pediatric Immunology and Allergy, İzmir, Türkiye

Tunca S, Aygün A. Childhood Mastocytosis, Nutrition and Food Allergy. In: Özdemir Ö, editor. Childhood Mastocytosis: New Developments in Diagnosis and Treatment. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.185-194.

ABSTRACT

Childhood mastocytosis is a rare group of disorders characterized by the abnormal accumulation and hyperactivation of mast cells. This condition leads to the excessive release of mediators such as histamine, tryptase, and cytokines, resulting in widespread symptoms affecting the skin, respirato- ry, gastrointestinal, and cardiovascular systems. Food allergies, mediated through IgE-dependent or non-IgE-dependent mechanisms, involve abnormal immune responses to dietary antigens. The co- existence of mastocytosis and food allergies exacerbates immune reactions and increases the risk of severe outcomes such as anaphylaxis, presenting significant clinical challenges. The shared patho- physiological basis between mastocytosis and food allergies is rooted in mast cell hyperreactivity. In IgE-mediated food allergies, mast cells are activated through allergen-specific IgE antibodies, whereas in mastocytosis, mast cells can degranulate spontaneously or in response to minimal stimuli. This hy- peractivation amplifies inflammatory responses, increases intestinal permeability, and triggers severe clinical symptoms. Non-IgE mechanisms, involving T-cell activation and chronic inflammation, fur- ther contribute to complications such as eosinophilic esophagitis and food protein-induced enterocoli- tis. Risk factors for food allergies in mastocytosis include atopic predisposition, an increased mast cell burden, and genetic factors, particularly mutations in the c-KIT gene. These factors enhance mast cell reactivity, intensifying immune responses. Although the prevalence of food allergies in children with mastocytosis remains underreported, evidence indicates a higher incidence and severity compared to the general population. Diagnosis requires a comprehensive clinical history, specific IgE testing, skin prick tests, and carefully conducted oral food challenges. Due to the increased risk of severe reactions, these diagnostic procedures must be performed under expert supervision in controlled settings. Serum tryptase levels serve as valuable biomarkers for assessing reaction severity. Management strategies necessitate a multidisciplinary approach, including the identification and elimination of trigger foods, pharmacological interventions (antihistamines, mast cell stabilizers, epinephrine), and dietary coun- seling to prevent nutritional deficiencies. Emerging treatments, such as omalizumab, offer promise for managing IgE-mediated food allergies. Additionally, patient and caregiver education, along with personalized emergency action plans, are essential components for improving patient outcomes and quality of life. In conclusion, the management of childhood mastocytosis and food allergies requires a detailed diagnostic process, individualized treatment plans, and innovative therapeutic approaches. This comprehensive and multidisciplinary strategy aims to enhance the quality of life and improve long-term clinical outcomes for affected patients.

Keywords: Mast cells; Food hypersensitivity; Mastocytosis; Intestinal mucosa; Diet; Food; Nutrition

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