REVIEWUpdates in diagnosis and management of paediatric mastocytosisTiano, Rasaa; Krase, Ifat Z.a,b; Sacco, Keitha,c Author Information aDivision of Pulmonology, Section of Allergy-Immunology, Phoenix Children's Hospital, Phoenix bDivison of Allergy-Immunology, Mayo Clinic, Scottsdale cDepartment of Child Health, University of Arizona College of Medicine, Phoenix, Arizona, USA Correspondence to Keith Sacco, MD, 1919 E. Thomas Rd. | Phoenix, AZ 85016, USA. Tel: +1 602 933 3889; e-mail: [email protected] Current Opinion in Allergy and Clinical Immunology: November 03, 2022 - Volume - Issue - 10.1097/ACI.0000000000000869 doi: 10.1097/ACI.0000000000000869 Buy PAP Metrics Abstract Purpose of review Paediatric mastocytosis is a rare clonal disorder characterized by the overproduction and organ infiltration of mast cells. Symptoms are due to mast cell mediator release. Cutaneous mastocytosis is the most common presentation in children with systemic disease being rare. Our aim is to provide a practical guideline in differentiating subtypes of paediatric mastocytosis while providing actionable recommendations on diagnosis, clinical management, follow-up and prognosis. Recent findings Longitudinal cohort studies of paediatric cutaneous mastocytosis have shown spontaneous remission with favourable prognosis. Hereditary alpha-tryptasemia may coexist with mastocytosis; thus, screening for this disorder is recommended. There is an emerging role for serum tryptase in asthma endotyping and potential for using therapeutic tryptase inhibitors. Summary Morbidity in paediatric mastocytosis typically arises from symptoms secondary to mast cell mediator release. Prognosis for nonaggressive disease is typically favourable; however, risks for anaphylaxis and psychosocial morbidity may be underestimated. Symptomatic management and anticipatory guidance may help support patients and families throughout the disease course. Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.