Systemic Mastocytosis in United States: A Population Based Study

Introduction: Systemic mastocytosis (SM) is a rare hematological disorder characterized by clonal proliferation and activation of abnormal mast cells. It can vary from an indolent form to an aggressive form including progression to leukemia. There is limited data on epidemiology, clinical characteristics and outcome of this disease in population based setting. Till date, a retrospective study by Lim, Ken-Hong, et.al which included 342 patients diagnosed with systemic mastocytosis in Mayo clinic, is the largest series of patient published so far. This study reports the clinical presentation and outcome of patients with SM but there are no population based study in United States so far. We therefore conducted this population based study to determine epidemiology, survival pattern and incidence of second primary malignancy among patients with SM. Methods: We used SEER 18 database (2000-2014) to select all adult patients with age 20 or above with SM. Patient population was divided into various cohorts based on age (20-59, 60-79 and 80+ years), sex, race (Caucasians, African American and Others), area of residence (rural, urban and metropolitan) and annual household income (