Table 1. Patients’ characteristics. Data of patients with indolent systemic mastocytosis (ISM), bone marrow mastocytosis (BMM), and smoldering systemic mastocytosis (SSM).
| Characteristics | Statistics/categories | ISM | BMM | SSM | P * | P ** |
|---|---|---|---|---|---|---|
| Age (y)(n = 1085) | Median (5. perc; 95. perc) | 45.0 (24.0; 67.0) | 50.0 (27.0; 70.0) | 52.0 (30.0; 73.0) | <.001 | <.001 |
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| Serum tryptase (ng/mL) (n = 1028) | Median (5. perc; 95. perc) | 35.0 (8.9; 183.0) | 27.0 (11.0; 128.0) | 200.0 (29.5; 575.0) | <.001 | <.001 |
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| WBC (/μL) (n = 1027) | Median (5. perc; 95. perc) | 6600.0 (4 160.0; 10 900.0) | 6 400.0 (3 900.0; 10 600.0) | 6900.0 (2 900.0; 12 600.0) | .397 | .940 |
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| Hemoglobin (g/dL) (n = 1032) | Median (5. perc; 95. perc) | 13.9 (12.0; 16.0) | 14.4 (12.3; 16.4) | 13.2 (10.4; 16.6) | <.001 | <.004 |
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| Platelets (×103/μL) (n = 1025) | Median (5. perc; 95. perc) | 269.0 (179.0; 396.0) | 247.0 (162.0; 380.0) | 230.0 (112.0; 409.0) | <.001 | <.001 |
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| Alkaline phosphatase (U/L) (n = 873) | Median (5. perc; 95. perc) | 75.0 (45.0; 146.0) | 71.8 (44.5; 129.0) | 114.0 (56.0; 340.0) | .133 | <.001 |
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| Gender (n = 1085) | Women | 427 (65.2%) | 160 (42.4%) | 31 (58.5%) | <.001 | .370 |
| Men | 228 (34.8%) | 217 (57.6%) | 22 (41.5%) | |||
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| Performance status (WHO) (n = 1085) | 0–Normal activity | 380 (58.0%) | 270 (71.6%) | 15 (28.3%) | <.001 | <.001 |
| 1–Symptoms, but fully ambulatory | 255 (38.9%) | 96 (25.5%) | 32 (60.4%) | |||
| 2–Symptoms, but in bed <50% of the day | 9 (1.4%) | 6 (1.6%) | 5 (9.4%) | |||
| 3–Needs to be in bed >50% of the day, but not bedridden | 5 (0.8%) | 3 (0.8%) | 0 (0.0%) | |||
| 5–Unknown | 6 (0.9%) | 2 (0.5%) | 1 (1.9%) | |||
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| Typical skin involvement (n = 1085) | Yes | 655 (100%) | 0 (0%) | 43 (81.1%) | <.001 | <.001 |
| No | 0 (0%) | 377 (100%) | 10 (18.9%) | |||
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| MC infiltrates in BM biopsy (n = 1085) | Yes | 561 (85.6%) | 284 (75.3%) | 50 (94.3%) | <.001 | 0.006 |
| No a | 68 (10.4%) | 77 (20.4%) | 0 (0.0%) | |||
| Not done a | 26 (4.0%) | 16 (4.2%) | 3 (5.7%) | |||
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| KIT mutation D816V in BM (n = 1081) | Yes | 428 (65.6%) | 274 (72.9%) | 34 (64.2%) | .614 | .636 |
| No | 77 (11.8%) | 44 (11.7%) | 4 (7.5%) | |||
| Not done | 147 (22.5%) | 58 (15.4%) | 15 (28.3%) | |||
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| Spleen (palpable) (n = 1056) | Yes | 23 (3.6%) | 15 (4.0%) | 27 (52.9%) | .735 | <.001 |
| No | 603 (95.1%) | 352 (94.9%) | 24 (47.1%) | |||
| Unknown | 8 (1.3%) | 4 (1.1%) | 0 (0.0%) | |||
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| Hepatomegaly (palpable) (n = 1054) | Yes | 28 (4.4%) | 19 (5.1%) | 21 (41.2%) | .644 | <.001 |
| No | 597 (94.3%) | 347 (93.8%) | 29 (56.9%) | |||
| Unknown | 8 (1.3%) | 4 (1.1%) | 1 (2.0%) | |||
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| Darier sign positive (n = 787) | Yes | 419 (67.0%) | 65 (53.7%) | 23 (56.1%) | 1.000 | .279 |
| No | 38 (6.1%) | 6 (5.0%) | 4 (9.8%) | |||
| Unknown | 168 (26.9%) | 50 (41.3%) | 14 (34.1%) | |||
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| Lymphadenopathy (n = 1047) | Yes | 7 (1.1%) | 8 (2.2%) | 8 (15.7%) | .189 | <.001 |
| No | 590 (93.4%) | 341 (93.7%) | 37 (72.5%) | |||
| Unknown | 35 (5.5%) | 15 (4.1%) | 6 (11.8%) | |||
Note: Dataset for overall survival.
Abbreviations: BM, bone marrow; MC, mast cell; WBC, white blood count.
a
Patients without MC infiltrates in the bone marrow biopsy or with no bone marrow biopsy had MCs found in the bone marrow blood smear; therefore, the diagnosis of ISM or BMM has been established.
*
ISM vs BMM.
**
ISM vs SSM.
Bold P-values are statisticly significant.