CHILDHOOD MASTOCYTOSIS NEW DEVELOPMENTS IN DIAGNOSIS AND TREATMENT

CHI775LDHOOD_MASTOCYTOSI775S_NEW_DEVELOPMENTS_IN_DI775AGNOSI775S_AND_TREATMENTKAPAK

CONSIDERATIONS IN CHILDHOOD MASTOCYTOSIS AND FOLLOW-UP

Yakup Canıtez

Bursa Uludağ University, Faculty of Medicine, Department of Pediatric Allergy, Bursa, Türkiye

Canıtez Y. Considerations in Childhood Mastocytosis and Follow-up. In: Özdemir Ö, editor. Childhood Mastocytosis: New Developments in Diagnosis and Treatment. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.117-137.

ABSTRACT

Mast cell diseases and mastocytosis conditions constitute a heterogeneous and variable group of dis- eases. Regarding the points to consider and follow-up in childhood mastocytosis, careful diagnosis and differential diagnosis (e.g., differentiation of cutaneous mastocytosis/systemic mastocytosis and exclu- sion of other diseases that need to be considered in the differential diagnosis) are especially important at the first onset of symptoms. In childhood mastocytosis, determination of the severity of the disease, monitoring of clinical findings and symptoms, and monitoring of skin findings and other symptoms in terms of remission (in addition, use of the SCORMA index for these purposes) are essential in the initial diagnosis and subsequent period. Prevention/management of symptom-triggering factors and appropriate treatment when necessary are essential. It is recommended that laboratory and imaging studies (complete blood count, some biochemistry tests, basal serum tryptase, abdominal ultrasonog- raphy, bone marrow biopsy, etc.) be performed when necessary, depending on symptoms and clinical findings, at the time of diagnosis and/or during the follow-up period. Mast cell activation syndrome is one of the most important issues to remember during diagnosis, differential diagnosis, and follow-up. Diagnosis and differential diagnosis of mast cell activation syndrome should be made carefully and an appropriate treatment approach should be provided. In general, the risk of anaphylaxis in child- hood-onset mastocytosis (especially in cutaneous mastocytosis) is lower than in adults. Prevention of possible anaphylactic reactions in childhood mastocytosis should be carefully provided with patient education and appropriate medical measures. Appropriate medical approaches should be provided in anesthesia or other various medical interventions. Serious side effects are rarely seen during anesthesia in children with cutaneous mastocytosis, whose disease is limited to the skin. Childhood cutaneous mastocytosis cases should be monitored for the possibility of systemic mastocytosis findings, which may be seen during follow-up, although this is unlikely. There is no definitive treatment option for mast cell diseases yet. Therefore, management should be multidisciplinary and primarily aimed at avoiding triggers and the risk of recurrence of symptoms. Although there are many developments in the field of diagnosis and treatment, some of the recommendations remain limited, considering the limited number of double-blind, placebo-controlled studies and therapeutic agents.

Keywords: Mastocytosis; Childhood; Cutaneous mastocytosis; Systemic mastocytosis; Follow-up

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