Clinical and demographic features of the study participants
The mean age of patients with mastocytosis and HCs were 42.80±10.47 and 41.03±9.03 years, respectively (p>0.05). There was no significant difference between the patients and the HCs regarding age, sex and clinical features which can possibly affect the presence of vascular diseases (Table 1).
According to WHO mastocytosis classification criteria, 8 (16.3%), 34 (69.4%), 6 (12.2%) and 1 (2.1%) patients had CM, ISM, advSM and SSM, respectively. Aggressive SM (ASM) (n:4, 8.2%) and systemic mastocytosis (SM) with an associated hematologic neoplasm (SM-AHN) (n:2, 4.1%) were considered within the advSM group. Polycythemia vera (n:1) and essential thrombocytosis (n:1) were accompanying SM-AHN. The median duration time of mastocytosis was 52 (min-max:11-264) months. Twenty-two (44.9 %) patients had history of anaphylaxis and the most common trigger of anaphylaxis was venom allergy (n=9, 18.4%). Idiopathic anaphylaxis, drug induced anaphylaxis and food induced anaphylaxis were seen in 6 (12.2%), 4 (8.2%) and 2 (4.1%) patients, respectively. Forty-two patients (85.7%) had c-Kit D816V mutation. In 37 (75.5%) patients (8 CM, 29 ISM patients), skin involvement was observed and the most common skin manifestation was urticaria pigmentosa (n=36, %73.5). Osteoporosis and osteosclerosis were observed in 11 (22.4%) and 4 (8.2%) patients, respectively. Median serum baseline tryptase level 34.36 (min-max:3.5-1648) kU/L and 3.00 (min-max:1.0-6.0) kU/L in patients with mastocytosis and HCs, respectively (p<0.001) However, median total IgE level were 26 (min-max: 2.3-480) µg/L and 17.60 (min-max:5-169) µg/L in patients with mastocytosis and HCs, respectively (p>0.05).