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).