CHILDHOOD MASTOCYTOSIS NEW DEVELOPMENTS IN DIAGNOSIS AND TREATMENT

CHI775LDHOOD_MASTOCYTOSI775S_NEW_DEVELOPMENTS_IN_DI775AGNOSI775S_AND_TREATMENTKAPAK

RISK AND MANAGEMENT OF ANAPHYLAXIS IN CHILDHOOD MASTOCYTOSIS

Tuba Karakurt

İstanbul Medeniyet University, Faculty of Medicine, Department of Pediatric Immunology and Allergy, İstanbul, Türkiye

Karakurt T. Risk and Management of Anaphylaxis in Childhood Mastocytosis. In: Özdemir Ö, editor. Childhood Mastocytosis: New Developments in Diagnosis and Treatment. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.195-207.

ABSTRACT

Mastocytosis encompasses a diverse set of conditions defined by the accumulation of abnormal mast cells (MCs) in various organs. These MCs are clonal, exhibiting atypical morphological and pheno- typic characteristics, and their proliferation can affect one or multiple organ systems. Anaphylaxis, a potentially fatal acute systemic hypersensitivity reaction, is characterized by excessive MC acti- vation and the subsequent release of various mediators. In children diagnosed with mastocytosis, the incidence of anaphylaxis can reach up to 9%. Children with widespread cutaneous involvement, elevated levels of serum tryptase, and boys face a significantly higher risk of anaphylaxis, particularly during blistering episodes. While Hymenoptera stings are the most common trigger of anaphylaxis in adults with mastocytosis, they are uncommon in children. The triggers of anaphylaxis in pediatric mastocytosis patients are foods, vaccines, anesthetics, sudden exposure to cold water, and Hymenop- tera stings. However, most cases classified as idiopathic. The best-proven MC activation marker is tryptase. Tryptase levels taken within 4 hours of a suspected MC activation event should be compared with serum baseline tryptase levels. The primary medication of anaphylaxis is epinephrine which is administered at a dose of 0.01 mg/kg (maximum dose of 0.5 mg) intramuscularly. Children with mastocytosis require regular follow up with a multidisciplinary approach by centers experienced in the management of the disease. For the long-term control and prevention of anaphylaxis, strategies focus on minimizing both the intensity and occurrence of acute episodes. The basis of long-term management is the education of patients and their caregivers. A mastocytosis identification document, containing information about acute management, prescribed emergency drugs, and medications to ei- ther avoid or use under medical oversight, could provide significant benefits for the patient All patients with mastocytosis and allergic reactions to Hymenoptera stings are recommended to undergo venom immunotherapy. Vaccines in children with mastocytosis should be administered in an experienced center and under observation for a certain period of time. Omalizumab has recently been recommend- ed for the management of severe mediator-related symptoms and the prevention of anaphylaxis in patients with mastocytosis.

Keywords: Childhood mastocytosis; Anaphylaxis; Risk factors; Triggers; Epinephrine

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