https://canadianhematologytoday.com/article/view/3-1-Lee
Canadian Hematology Today, Volume 3, Issue 1, Spring 2024 / DOI: https://doi.org/10.58931/cht.2024.3149
Stephanie Lee, MD, MSc, FRCPC Division of Hematology/Oncology, St. Michael’s Hospital, Toronto, ON
Abstract
Mastocytosis is a group of clonal disorders characterized by an accumulation of neoplastic mast cells (MCs) in one or more organ systems. The clinical presentation of mastocytosis is heterogenous as are the clinical outcomes. For example, some variants are associated with near normal life expectancy, while others are amongst the most aggressive known malignancies. Mastocytosis can occur in both pediatric and adult populations and can be classified into three major groups: systemic mastocytosis (SM), cutaneous mastocytosis (CM), and localized mast cell sarcoma. This review will focus on SM in adults with the aim of providing a general overview of the (1) pathophysiology, (2) diagnostic approach, and (3) current treatment landscape in Canada.
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