Current Guidelines and Management of Non-Advanced Systemic Mastocytosis

Published: Dec. 21, 2022, 5 a.m.

b'Guest: Matthew P. Giannetti, MD
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Systemic mastocytosis (SM) is a rare heterogeneous disease characterized by clonal expansion and accumulation of neoplastic mast cells in one or more organs (most commonly bone marrow with or without skin involvement). Most patients with SM have non-advanced disease (indolent and smoldering subtypes) for which there are currently no FDA-approved therapies that address the underlying pathogenesis of the disease. Given its rarity, many clinicians are not aware of the unique clinical features, disease burden, or the recent updates to the World Health Organization (WHO) diagnostic and subtyping criteria for SM, resulting in long delays in diagnosis and suboptimal care in clinical practice. Taken together, these practice gaps highlight a need for evidence-based, up-to-date continuing education activities for clinicians on optimal diagnosis and management of non-advanced SM.

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