CHILDHOOD MASTOCYTOSIS NEW DEVELOPMENTS IN DIAGNOSIS AND TREATMENT

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CHILDHOOD MASTOCYTOSIS AND DRUG ALLERGY

Ece Şenbaykal Yiğit1
Sanem Eren Akarcan2
Tuba Tuncel3

1İzmir City Hospital, Department of Pediatric Immunology and Allergy, İzmir, Türkiye
2İzmir City Hospital, Department of Pediatric Immunology and Allergy, İzmir, Türkiye
3izmir Katip Çelebi University, Faculty of Medicine, Department of Pediatric Immunology and Allergy, İzmir, Türkiye

Şenbaykal Yiğit E, Eren Akaracan S, Tuncel T. Childhood Mastocytosis and Drug Allergy. In: Özdemir Ö, editor. Childhood Mastocytosis: New Developments in Diagnosis and Treatment. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.159-169.

ABSTRACT

Mastocytosis is a heterogeneous group of diseases characterized by clonal proliferation of mast cells in one or more organs or systems. Cutaneous mastocytosis is the most common form in childhood. Anaphylaxis, drug allergy, and similar allergic reactions are more common in patients with mastocy- tosis than in the normal population due to the increase in mast cell burden and mediator release from mast cells. Since systemic mastocytosis is rare in children, the frequency of these reactions in pediatric mastocytosis cases is not as high as in adults. But the frequency increases in children with widespread cutaneous mastocytosis lesions, bullous cutaneous disease, and high blood tryptase levels. Non-steroi- dal anti-inflammatory drugs, antibiotics, anesthetic agents, opioids, and radiocontrast media are among the drugs that have been reported to cause drug hypersensitivity reactions and anaphylaxis in patients with mastocytosis. Drug hypersensitivity reactions begin through four different receptor types (c-KIT, MRGPRX2, FceRI, C5aR/CD88) located on the mast cell surface with IgE-mediated and non-IgE-me- diated mechanisms. Additionally, cyclooxygenase enzyme inhibition by non-steroidal anti-inflammato- ry drugs and paracetamol potentiates the stimulation of mast cell receptors via increased leukotrienes. From activated mast cells, histamine and tryptase are released at first, followed by leukotrienes and some other mediators, potentiating the reaction further. In each drug group, the drugs with a higher risk of reaction and those that are safer have been determined based on both reaction mechanisms and clinical experiences. In patients with mastocytosis, the use of drugs with a high risk of mast cell acti- vation should be avoided. If no alternative medication is available, premedication or when necessary desensitization should be considered.

Keywords: Anesthetic agents; Anti-bacterial agents; Childhood; Drug hypersensitivity; Mast cells; Mastocytosis; Non-steroidal anti-inflammatory agents

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