Table 2. Patients’ characteristics. Data of patients with indolent systemic mastocytosis (ISM), cutaneous mastocytosis (CM), adulthood CM (aCM) and mastocytosis in the skin (MIS).
| Characteristics | Statistics/categories | ISM | CM | aCM | MIS | P * | P ** | P *** |
|---|---|---|---|---|---|---|---|---|
| Age (yrs) (n = 1108) | Median (5. perc; 95. perc) | 45.0 (24.0; 67.0) | 23.0 (0.6; 60.0) | 37.0 (22.0; 60.0) | 42.0 (17.0; 69.0) | <.001 | <.001 | .013 |
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| Serum tryptase (ng/mL) (n = 1007) | Median (5. perc; 95. perc) | 35.0 (8.9; 183.0) | 7.6 (2.7; 27.8) | 9.2 (2.7; 26.9) | 12.0 (2.7; 64.0) | <.001 | <.001 | <.001 |
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| WBC (/μL) (n = 966) | Median (5. perc; 95. perc) | 6 600.0 (4 160.0; 10 900.0) | 7 000.0 (4 510.0; 12 800.0) | 6300.0 (4 510.0; 12 800.0) | 6 690.0 (4 410.0; 11 000.0) | .025 | .484 | .343 |
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| Hemoglobin (g/dL) (n = 975) | Median (5. perc; 95. perc) | 13.9 (12.0; 16.0) | 13.5 (10.8; 15.9) | 13.9 (12.1; 15.9) | 13.8 (11.7; 15.8) | <.001 | .807 | .194 |
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| Platelets (×103/μL) (n = 967) | Median (5. perc; 95. perc) | 269.0 (179.0; 396.0) | 276.5 (173.0; 434.0) | 258.0 (173.0; 386.0) | 254.0 (183.0; 390.0) | .022 | .081 | .231 |
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| Alkaline phosphatase (U/L) (n = 754) | Median (5. perc; 95. perc) | 75.0 (45.0; 146.0) | 76.0 (40.0; 357.0) | 68.0 (40.0; 148.0) | 66.0 (42.0; 135.0) | .126 | .063 | .014 |
| Gender (n = 1108) | Women | 427 (65.2%) | 143 (59.1%) | 92 (69.2%) | 137 (64.9%) | .101 | .423 | 1.000 |
| Men | 228 (34.8%) | 99 (40.9%) | 41 (30.8%) | 74 (35.1%) | ||||
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| Performance status (WHO) (n = 1106) | 0–Normal activity | 380 (58.0%) | 166 (68.9%) | 79 (59.8%) | 153 (72.9%) | .007 | .409 | .001 |
| 1–Symptoms, but fully ambulatory | 255 (38.9%) | 74 (30.7%) | 52 (39.4%) | 53 (25.2%) | ||||
| 2–Symptoms, but in bed <50% of the day | 9 (1.4%) | 0 (0.0%) | 0 (0.0%) | 2 (1.0%) | ||||
| 3–Needs to be in bed >50% of the day, but not bedridden | 5 (0.8%) | 0 (0.0%) | 0 (0.0%) | 0 (0.0%) | ||||
| 4–Unable to get out of bed | 0 (0.0%) | 0 (0.0%) | 0 (0.0%) | 1 (0.5%) | ||||
| 5–Unknown | 6 (0.9%) | 1 (0.4%) | 1 (0.4%) | 1 (0.5%) | ||||
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| MC infiltrates in BM biopsy (n = 1108)a | Yes | 561 (85.6%) | 2 (0.8%) | 2 (1.5%) | 0 (0.0%) | <.001 | <.001 | <.001 |
| No | 68 (10.4%) | 133 (55.0%) | 128 (96.2%) | 10 (4.7%) | ||||
| Not done | 26 (4.0%) | 107 (44.2%) | 3 (2.3%) | 201 (95.3%) | ||||
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| C-kit mutation D816V in BM (n = 1103) | Yes | 428 (65.6%) | 24 (10.0%) | 24 (18.3%) | 7 (3.3%) | <.001 | <.001 | <.001 |
| No | 77 (11.8%) | 81 (33.8%) | 79 (60.3%) | 9 (4.3%) | ||||
| Not done | 147 (22.5%) | 135 (56.3%) | 28 (21.4%) | 195 (92.4%) | ||||
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| Spleen (palpable) (n = 1053) | Yes | 23 (3.6%) | 3 (1.3%) | 3 (2.3%) | 0 (0.0%) | .113 | .600 | .007 |
| No | 603 (95.1%) | 208 (93.3%) | 125 (94.7%) | 165 (84.2%) | ||||
| Unknown | 8 (1.3%) | 12 (5.4%) | 4 (3.0%) | 31 (15.8%) | ||||
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| Hepatomegaly (palpable) (n = 1051) | Yes | 28 (4.4%) | 8 (3.6%) | 7 (5.3%) | 6 (3.1%) | .845 | .644 | .829 |
| No | 597 (94.3%) | 202 (91.0%) | 121 (91.7%) | 159 (81.1%) | ||||
| Unknown | 8 (1.3%) | 12 (5.4%) | 4 (3.0%) | 31 (15.8%) | ||||
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| Darier sign positive (n = 1069) | Yes | 419 (67.0%) | 178 (74.8%) | 85 (64.9%) | 141 (68.4%) | .761 | .240 | .337 |
| No | 38 (6.1%) | 18 (7.6%) | 12 (9.2%) | 17 (8.3%) | ||||
| Unknown | 168 (26.9%) | 42 (17.6%) | 34 (26.0%) | 48 (23.3%) | ||||
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| Lymphadenopathy (n = 1043) | Yes | 7 (1.1%) | 7 (3.2%) | 2 (1.6%) | 11 (5.8%) | .052 | .657 | <.001 |
| No | 590 (93.4%) | 188 (85.5%) | 122 (94.6%) | 138 (72.3%) | ||||
| Unknown | 35 (5.5%) | 25 (11.4%) | 5 (3.9%) | 42 (22.0%) | ||||
Note: Dataset for overall survival.
Abbreviations: BM, bone marrow; MC, mast cell; NA, not applicable; WBC, white blood count.
a
The diagnosis ISM can be established based on the presence of three minor criterion even in the absence of the major criterion (dense mast cell infiltrate); not every mast cell infiltrate is diagnostic even if it is clearly visible; a loose infiltrate without densely packed atypical mast cells does not qualify as a major SM criterion.
*
ISM vs CM.
**
ISM vs aCM.
***
ISM vs MIS.
Bold P-values are statisticly significant.