Laboratory and clinical characteristics of CALR mutant patients compared with JAK2V617F or MPLW515 mutant patients and patients who were wt for the 3 mutations
| . | CALR+ . | JAK2 V617F+ . | MPL W515+ . | CALR, JAK2, MPL wt . | P value . | ||
|---|---|---|---|---|---|---|---|
| CALR+ vs JAK2 V617F+ . | CALR+ vs MPL W515+ . | CALR+ vs CALR, JAK2, MPL wt . | |||||
| Number of patients (%) | 89 (15.5) | 369 (64.1) | 25 (4.3) | 93 (16.1) | — | — | — |
| Male, no. (%) | 53 (59.5) | 117 (31.7) | 6 (24.0) | 18 (19.4) | <0.0001 | 0.002 | <0.0001 |
| Age, years | 54.7 (13-88) | 61 (15-93) | 54 (22.89) | 53 (15-87) | 0.04 | 0.997 | 0.519 |
| Leukocyte count (×109/L) | 8.1 (3.5-26.0) | 8.9 (4.2-35.0) | 8.4 (4.5-16.6) | 8.3 (4-16.8) | 0.001 | 0.834 | 0.367 |
| Hemoglobin (g/L) | 138 (106-173) | 145 (102-173) | 136 (110-160) | 136 (106-164) | <0.0001 | 0.315 | 0.380 |
| Hematocrit (%) | 41.2 (35.9-49.4) | 43.8 (31.4-53.6) | 41.2 (32.8-50) | 41.0 (31.3-51.5) | <0.0001 | 0.887 | 0.893 |
| Platelet count (×109/L) | 866 (504-2348) | 726 (455-1881) | 898 (607-2000) | 697 (482-1659) | <0.0001 | 0.385 | <0.0001 |
| Lactate dehydrogenase (U/L) | 320 (142-725) | 288 (102-1178) | 365 (254-570) | 268 (137-554) | 0.307 | 0.665 | <0.01 |
| Splenomegaly, no. (%) | 24 (27.0) | 91 (24.7) | 9 (36.0) | 9 (9.7) | 0.661 | 0.416 | 0.004 |
| Pruritus, no. (%) | 5 (5.6) | 32 (8.7) | 1 (4.0) | 9 (9.7) | 0.260 | 0.847 | 0.228 |
| Constitutional symptoms, no. (%) | 1 (1.1) | 19 (5.1) | 1 (1.2) | 6 (6.5) | 0.120 | 0.577 | 0.078 |
| Major thrombosis, no. (%) | 12 (13.5) | 111 (30.1) | 10 (40.0) | 15 (16.1) | 0.011 | 0.012 | 0.894 |
| Microvessel symptoms, no. (%) | 22 (24.7) | 101 (27.4) | 14 (56.0) | 20 (21.5) | 0.604 | 0.003 | 0.674 |
| Major hemorrhage, no. (%) | 4 (4.5) | 17 (4.6) | 4 (16.0) | 3 (3.3) | 0.906 | 0.067 | 0.587 |
| Progression to PET-MF, no. (%) | 4 (4.5) | 12 (3.3) | 2 (8.0) | 1 (1.1) | 0.458 | 0.563 | 0.128 |
| Progression to PV, no. (%) | 0 | 5 (1.4) | 0 | 0 | 0.294 | — | — |
| Progression to AL, no. (%) | 0 | 2 (0.5) | 1 (4.0) | 1 (1.1) | 0.507 | 0.071 | 0.349 |
| Deceased (n = 70) (%) | 10 (11.2) | 49 (13.3) | 4 (16.0) | 7 (7.5) | 0.598 | 0.515 | 0.414 |
| Cytoreductive therapy, no. (%) | 50 (62.5) | 220 (60.8) | 19 (76.0) | 40 (46.0) | 0.440 | 0.160 | 0.023 |
| . | CALR+ . | JAK2 V617F+ . | MPL W515+ . | CALR, JAK2, MPL wt . | P value . | ||
|---|---|---|---|---|---|---|---|
| CALR+ vs JAK2 V617F+ . | CALR+ vs MPL W515+ . | CALR+ vs CALR, JAK2, MPL wt . | |||||
| Number of patients (%) | 89 (15.5) | 369 (64.1) | 25 (4.3) | 93 (16.1) | — | — | — |
| Male, no. (%) | 53 (59.5) | 117 (31.7) | 6 (24.0) | 18 (19.4) | <0.0001 | 0.002 | <0.0001 |
| Age, years | 54.7 (13-88) | 61 (15-93) | 54 (22.89) | 53 (15-87) | 0.04 | 0.997 | 0.519 |
| Leukocyte count (×109/L) | 8.1 (3.5-26.0) | 8.9 (4.2-35.0) | 8.4 (4.5-16.6) | 8.3 (4-16.8) | 0.001 | 0.834 | 0.367 |
| Hemoglobin (g/L) | 138 (106-173) | 145 (102-173) | 136 (110-160) | 136 (106-164) | <0.0001 | 0.315 | 0.380 |
| Hematocrit (%) | 41.2 (35.9-49.4) | 43.8 (31.4-53.6) | 41.2 (32.8-50) | 41.0 (31.3-51.5) | <0.0001 | 0.887 | 0.893 |
| Platelet count (×109/L) | 866 (504-2348) | 726 (455-1881) | 898 (607-2000) | 697 (482-1659) | <0.0001 | 0.385 | <0.0001 |
| Lactate dehydrogenase (U/L) | 320 (142-725) | 288 (102-1178) | 365 (254-570) | 268 (137-554) | 0.307 | 0.665 | <0.01 |
| Splenomegaly, no. (%) | 24 (27.0) | 91 (24.7) | 9 (36.0) | 9 (9.7) | 0.661 | 0.416 | 0.004 |
| Pruritus, no. (%) | 5 (5.6) | 32 (8.7) | 1 (4.0) | 9 (9.7) | 0.260 | 0.847 | 0.228 |
| Constitutional symptoms, no. (%) | 1 (1.1) | 19 (5.1) | 1 (1.2) | 6 (6.5) | 0.120 | 0.577 | 0.078 |
| Major thrombosis, no. (%) | 12 (13.5) | 111 (30.1) | 10 (40.0) | 15 (16.1) | 0.011 | 0.012 | 0.894 |
| Microvessel symptoms, no. (%) | 22 (24.7) | 101 (27.4) | 14 (56.0) | 20 (21.5) | 0.604 | 0.003 | 0.674 |
| Major hemorrhage, no. (%) | 4 (4.5) | 17 (4.6) | 4 (16.0) | 3 (3.3) | 0.906 | 0.067 | 0.587 |
| Progression to PET-MF, no. (%) | 4 (4.5) | 12 (3.3) | 2 (8.0) | 1 (1.1) | 0.458 | 0.563 | 0.128 |
| Progression to PV, no. (%) | 0 | 5 (1.4) | 0 | 0 | 0.294 | — | — |
| Progression to AL, no. (%) | 0 | 2 (0.5) | 1 (4.0) | 1 (1.1) | 0.507 | 0.071 | 0.349 |
| Deceased (n = 70) (%) | 10 (11.2) | 49 (13.3) | 4 (16.0) | 7 (7.5) | 0.598 | 0.515 | 0.414 |
| Cytoreductive therapy, no. (%) | 50 (62.5) | 220 (60.8) | 19 (76.0) | 40 (46.0) | 0.440 | 0.160 | 0.023 |
Hematologic and clinical information was collected at diagnosis; information regarding major thrombosis and hemorrhage included events at diagnosis, in the 2 preceding years, and during follow-up. Cytoreduction means that the patient received cytoreductive drugs (in >90% of cases, this was hydroxyurea) during the course of the disease at the physician’s discretion, based on conventional criteria. Unless otherwise indicated, values are reported as median (range). Statistically significant differences are shown in bold.