Table 2.
Clinical characteristics of all patients, molecular data and karyotype
| Pt. Characteristics | PMF, N= 755 | PET/MF, N=163 | PPV-MF, N=181 |
|---|---|---|---|
| JAK2 positive, N (% of eval.) | 369/442 (83) | 65/99 (66) | 157/157 (100) |
| Median JAK2 allele frequency, % (range) | 47 (1–99) | 58 (7–96) | 86 (5–99)* |
| MPL positive, N (%) | 22 (5) | 7 (7) | -- |
| CALR positive, N (%) | 32 (7) | 23 (23) | -- |
| Triple negative, N (%) | 21 (5) | 4 (4) | -- |
| HMR mutations, N (%) | 45/198 (22) | 7/38 (18) | 15/147 (10) |
| DIPSS – low, N (%) | 67 (9) | 9 (6) | 16 (9) |
| Int-1, N (%) | 261 (35) | 68 (42) | 69 (38) |
| Int-2, N (%) | 325 (43) | 80 (49) | 55 (31) |
| High, N (%) | 96 (13) | 23 (14) | 23 (13) |
| Advanced BM fibrosis, N (%) | 588/667 (88) | 150/162 (93) | 145/149 (97)* |
| Karyotype, total eval., N | N = 660 | N= 113 | N=208 |
| Diploid, N (%/) | 429 (65) | 56 (50) | 136 (65) |
| Single Abn, N (%) | 144 (22) | 44 (39) | 31 (15) |
| Single Deletion 13q / 20q, N | 75 | 17 | 21 |
| Deletion 7/7q, 5/5q, 12p-, INV (3), Abn 17, N | 18 | 3 | 2 |
| All Others**, N | 51 | 24 | 8 |
| Double Abn, N (%) | 54 (8) | 5 (4) | 20 (10) |
| Including Abn 5, 7, 12p, 11q23, 17, N | 16 | -- | 3 |
| Including Abn 13q, 20q, N | 10 | 3 | 2 |
| All Others**, N | 28 | 2 | 15 |
| Complex (≥3 Abn), N (%) | 33 (5) | 8 (7) | 21 (10) |
*
Statistically significant differences (p<0.05) are shown in bold; Advanced bone marrow fibrosis = Grade 2 or higher according to European grading; HMR mutations = ASXL1, IDH1, IDH2, EZH2;
**
Ab = single or combinations of: INV, DER and TRANSL. of 1, 3, 6, 8, 9, 12, 13, 15, 18 and Y; trisomy of 1, 8, 9, 21, 13; ADD of 21, 2; DEL 6p, 8p, 1p, 13q, 20q, Y; 131 patients with PMF and 39 patients with PET-MF who were negative for JAK2 and MPL, but not tested for CALR are not included in the table.