| Gender (males: females) |
7:12 |
| Age (years old) |
47 [17-83] |
| Presenting features |
|
| Age at onset (years)* |
35 [0.67-62] |
| Age at diagnosis (years)† |
44 [15-70] |
| Time between the onset and diagnosis (years) |
16 [3-48] |
| Follow-up from diagnosis (years)§ |
3 [0.6-18] |
| Disease manifestations |
|
| Pruritus |
14 (74%) |
| Cutaneous |
19 (100%) |
| Gastrointestinal |
16 (84%) |
| Osteoarticular |
14 (74%) |
| Neuropsychiatric |
9 (47%) |
| Allergic |
10 (53%) |
| Cardiovascular |
9 (47%) |
| Skin lesions |
|
| Generalized |
2 (11%) |
| Restricted to one area |
6 (32%) |
| More than one area involved, but not generalized |
11 (58%) |
| Laboratory findings at presentation |
|
| Total IgE (KU/L)‡ |
10.3 [<2-231] |
| Tryptase (mg/L)§ |
24.9 [4-305] |
|
Results are expressed as median [range] values, absolute numbers and
(percentages).
*Date of the first mast cell mediators release episode or appearance of skin lesions
†Date of the first bone marrow study or skin biopsy
‡Normal values: IgE<100 KU/L
§Normal values: Tryptase<13.5 μg/L |
| Table 2: Clinical and laboratory characteristics from the patients included in this
study. |
