Table 1.
Baseline clinical, laboratory, genetic, and outcome characteristics of patients with indolent and advanced systemic mastocytosis
| Characteristics | ISM (n = 29) | AdvSM (n = 32) | P |
|---|---|---|---|
| Age (years) | |||
| Median | 42 | 70 | < 0.0001 |
| Range | 28–74 | 28–82 | |
| Sex, n (%) | |||
| Men | 12 (41) | 21 (66) | NS |
| Women | 17 (59) | 11 (34) | NS |
| Hemoglobin (g/dL) | |||
| Median | 14.1 | 10.9 | < 0.0001 |
| Range | 12–15.8 | 7.5–14,8 | |
| < 10 g/dL, n (%) | 0 (0) | 13 (41) | < 0.0001 |
| Platelets (× 109/L) | |||
| Median | 263 | 114 | < 0.0001 |
| Range | 124–497 | 39–577 | |
| < 100 × 109/L, n (%) | 0 (0) | 13 (41) | < 0.0001 |
| Mast cell infiltration in BM histology (%) | |||
| Median | 10 | 30 | 0.0003 |
| Range | 5–40 | 5–95 | |
| Serum tryptase (µg/L) | |||
| Median | 36 | 211 | < 0.0001 |
| Range | 8–106 | 14–1250 | |
| Alkaline phosphatase (U/L) | |||
| Median | 70 | 190 | < 0.0001 |
| Range | 40–244 | 55–756 | |
| > UNL, n (%) | 1 (3) | 22 (69) | < 0.0001 |
| Splenomegaly, n (%) | 4 (14) | 30 (94) | < 0.0001 |
| KIT D816V positive | 26 (90) | 31 (97) | NS |
| Follow-up (years) | |||
| Median | 12.1 | 3.6 | |
| Range | 0.1–29.2 | 0.1–19.3 | |
| Overall survival (years) | |||
| Median | NR | 3.8 | < 0.0001 |
| 95% CI | – | 2.9–4.7 | |
| Death, n (%) | 0 (0) | 21 (66) | |
AdvSM advanced systemic mastocytosis, BM bone marrow, CI confidence interval, ISM indolent systemic mastocytosis, NR not reached, NS non-significant, UNL upper normal limit, WHO World Health Organization