Systemic Mastocytosis in Adults: Long-Term Follow up and Real-Life Experience

Abstract

Systemic mastocytosis (SM) is a rare disease and its clinical presentations and life expectancy are defined in previous studies. However, the real-life experience in SM from developing countries, especially with long term follow up data is limited. Patients who were diagnosed with SM were enrolled and defined as SM subgroups. Indolent SM (ISM)/ smoldering SM (SSM) and advanced SM (advSM) patients were compared according to clinical and laboratory findings. 22 SM patients with the median age was 42 years were evaluated. Eight of 22 (36.4%) patients had ISM, 4/22 (18.2%) had SSM, 5 had aggressive SM (ASM) and the remaining 5 had SM associated with AHN (SM-AHN). Median tryptase level was 61 ng/mL (range 10–200 ng/mL). Alkaline phosphatase level is higher in advSM than ISM/ SSM group (p= 0.003). Anaphylactic events were more frequent in ISM/SSM than advSM group: 9/12 (75%) versus 2/10 (20%), respectively (p= 0.03). Neurological findings are seen more commonly in ISM/SSM (83.3% vs 30%, p= 0.017). Headache and cognitive difficulties were noted in respectively 50% and 32% of patients. Overall survival (OS) was shorter in advASM group; OS at 2-year were 46.7% in advSM and %100 in ISM/SSM patients. Our study highlights the need of collaboration of different disciplines to improve the diagnostic approach and management of SM patients. Keyorwds: Systemic mastocytosis, Smoldering, Aggressive, Tryptase