Systemic mastocytosis: demographics and clinical characteristics of the study population according to disease category
Category . | No. of patients . | Median age, y (range) . | Sex, M/F . | Median WBC count, × 109/L (range) . | Median BM mast cells, % (range) . | UP, % . | Hepatomegaly, % . | Splenomegaly, % . | Eosinophilia, % . | KIT mutation test before treatment, % . |
|---|---|---|---|---|---|---|---|---|---|---|
| MCL | 6 | 69 (37-76) | 4/2 | 4.7 (1.5-13.4) | 36 (25-60) | 0 | 67 | 67 | 50 | 100 |
| ISM | 74 | 45 (21-79) | 34/40 | 6.2 (4.3-15.7) | 0.14 (0.013-1.65) | 99 | 5.4 | 4 | 14 | 100 |
| SM-ana | 7 | 46 (19-70) | 5/2 | 4.9 (3.9-8.2) | 0.15 (0.035-0.26) | 0 | 0 | 0 | 0 | 100 |
| WDSM | 7 | 23 (12-73) | 2/5 | 5.6 (4.0-6.2) | 0.30 (0.008-12) | 100 | 0 | 0 | 0 | 100 |
| ASM | 6 | 55 (25-77) | 5/1 | 4.8 (2.7-20.1) | 1.85 (0.02-19) | 33 | 83 | 100 | 0 | 67* |
| SM-AHNMD | 13 | 72 (37-83) | 8/5 | 6.1 (1.8-21.3) | 0.60 (0.013-7.4) | 62 | 23 | 23 | 23 | 92* |
Category . | No. of patients . | Median age, y (range) . | Sex, M/F . | Median WBC count, × 109/L (range) . | Median BM mast cells, % (range) . | UP, % . | Hepatomegaly, % . | Splenomegaly, % . | Eosinophilia, % . | KIT mutation test before treatment, % . |
|---|---|---|---|---|---|---|---|---|---|---|
| MCL | 6 | 69 (37-76) | 4/2 | 4.7 (1.5-13.4) | 36 (25-60) | 0 | 67 | 67 | 50 | 100 |
| ISM | 74 | 45 (21-79) | 34/40 | 6.2 (4.3-15.7) | 0.14 (0.013-1.65) | 99 | 5.4 | 4 | 14 | 100 |
| SM-ana | 7 | 46 (19-70) | 5/2 | 4.9 (3.9-8.2) | 0.15 (0.035-0.26) | 0 | 0 | 0 | 0 | 100 |
| WDSM | 7 | 23 (12-73) | 2/5 | 5.6 (4.0-6.2) | 0.30 (0.008-12) | 100 | 0 | 0 | 0 | 100 |
| ASM | 6 | 55 (25-77) | 5/1 | 4.8 (2.7-20.1) | 1.85 (0.02-19) | 33 | 83 | 100 | 0 | 67* |
| SM-AHNMD | 13 | 72 (37-83) | 8/5 | 6.1 (1.8-21.3) | 0.60 (0.013-7.4) | 62 | 23 | 23 | 23 | 92* |
UP indicates urticaria pigmentosa.
In those 3 patients tested at time of therapy (1 SM-AHNMD and 2 ASM), all cell populations tested were positive for the D816V KIT mutation.