Table 1.
Demographics and description of all 3403 patients according to the WHO categories
| Mastocytoma | DCM | MPCM | MIS | ISM | SSM | ASM | SM-AHN | MCL | |
|---|---|---|---|---|---|---|---|---|---|
| Number (%) of patients at diagnosis | 91 (2.7%) | 49 (1.4%) | 502 (14.8%) | 524 (15.4%) | 1730 (50.8%) | 69 (2.0%) | 112 (3.3%) | 283 (8.3%) | 43 (1.3%) |
| % Male | 59 | 51 | 44 | 33 | 44 | 44 | 52 | 70 | 58 |
| Age at diagnosis in years; median (range) | |||||||||
| Male | 1 (0-5) | 2 (0-59) | 3 (0-82) | 44 (19-85) | 48 (0-82) | 61 (30-78) | 63 (16-82) | 66 (1-87) | 60 (35-91) |
| Female | 2 (0-15) | 8 (0-68) | 26 (0-78) | 40 (18-87) | 47 (15-83) | 52 (25-79) | 57 (29-83) | 66 (20-87) | 51 (27-72) |
| Tryptase, median (range) | 5.9 (1.3-26.4) | 15.1 (1.7-103) | 7.1 (1-126) | 13.8 (1-200) | 30 (1-885) | 200 (21-2100) | 165 (8.9-1432) | 135 (1.8-1060) | 383 (74.9-4530) |
| Number of patients with follow-up data | 49 | 32 | 324 | 277 | 1262 | 61 | 88 | 228 | 36 |
Abbreviations: DCM: diffuse cutaneous mastocytosis; MPCM: maculopapular cutaneous mastocytosis; MIS: mastocytosis in the skin; ISM: indolent systemic mastocytosis; SSM: smoldering systemic mastocytosis; ASM: aggressive systemic mastocytosis; SM-AHN: systemic mastocytosis with an associated hematologic neoplasm; MCL, mast cell leukemia; WHO, World Health Organization.