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Mastocytosis is a term encompassing a group of clinical disorders characterised by clonal proliferation of abnormal mast cells (MCs) in organ systems of the body. Mastocytosis can be systemic (with or without skin involvement) or cutaneous, and can affect organs including bone marrow, liver, spleen, lymph nodes and mucosal surfaces. Patients with systemic mastocytosis (SM) are susceptible to triggers that could cause activation of abnormal MCs, resulting in multiorgan dysfunction and life-threatening anaphylactic reactions. Mastocytosis has a number of ramifications for the dental management of a patient with the condition. Patients are at increased risk of complications due to a number of risk factors for MC activation present within the dental context, including stress, certain prescribed drugs, oral hygiene products and dental materials. This report presents the oral management of an adult with SM, discussing the implications of the condition within the context of the limited existing literature on the subject.

Mast cell disorders include mastocytosis and mast cell activation syndromes. Mastocytosis is a rare clonal disorder of the mast cell, driven by KIT D816V mutation in most cases. Mastocytosis is diagnosed and classified according to World Health Organization criteria. Mast cell activation syndromes encompass a diverse group of disorders and may have clonal or nonclonal etiologies. Hematologists may be consulted to assist in the diagnostic workup and/or management of mast cell disorders. A consult to the hematologist for mast cell disorders may provoke anxiety due to the rare nature of these diseases and the management of nonhematologic mast cell activation symptoms. This article presents recommendations on how to approach the diagnosis and management of patients referred for common clinical scenarios.

[No Abstract Available]

Advanced Systemic Mastocytosis (Adv-SM) is rare and has a poor prognosis. Midostaurin (Rydapt