Patient characteristics and treatment information according to disease type (n = 103)
| Variable . | ET . | PV . | MF . | PMF . | post ET-MF . | post PV-MF . |
|---|---|---|---|---|---|---|
| No. of patients per subgroup | 47 | 30 | 28 | 18 | 9 | 1 |
| Sex | ||||||
| Female | 28 | 14 | 17 | |||
| Male | 19 | 16 | 11 | |||
| Median age, y | 64.6 | 65.3 | 67.7 | 60.6 | 66.1 | 76.5 |
| Median duration of disease, y | 11.5 | 6.2 | 11 | 5.1 | 22.0 | 4.8 |
| Median MPN Symptom Assessment Form total symptom score | 11.0 | 15.5 | 8.0 | |||
| ECOG PS | ||||||
| 0 | 39 | 27 | 24 | |||
| 1 | 4 | 3 | 2 | |||
| 2 | 4 | 0 | 1 | |||
| Mutational status* | ||||||
| JAK2V617F and exon 12 | 31 | 30 | 19 | 12 | 6 | 1 |
| Type I and II CALR | 10 | 0 | 7 | 5 | 2 | 0 |
| MPL | 0 | 0 | 2 | 2 | 0 | 0 |
| Triple negative | 6 | 0 | 1 | 0 | 1 | 0 |
| No. of patients with vascular events | 11 (23.4) | 9 (30) | 8 (28.6) | 5 | 3 | 0 |
| Arterial | 9 | 7 | 7 | 2 | 2 | 0 |
| Venous | 11 | 7 | 7 | 5 | 2 | 0 |
| Splanchnic | 3 | 2 | 2 | 2 | 0 | 0 |
| Bleeding | 1 | 0 | 0 | |||
| Risk scores | ||||||
| IPSET-T | NA | NA | ||||
| Low | 10 | |||||
| Intermediate | 11 | |||||
| High | 26 | |||||
| ELN | NA | |||||
| Low | 10 | 7 | ||||
| High | 37 | 23 | ||||
| IPSET | NA | NA | ||||
| Low | 10 | |||||
| Intermediate | 30 | |||||
| High | 7 | |||||
| IPSS | NA | (unknown 4) | ||||
| Low | 10 | 16 | ||||
| Intermediate-1 | 5 | 5 | ||||
| Intermediate-2 | 11 | 6 | ||||
| High | 0 | |||||
| DIPSS | NA | NA | ||||
| Low | 13 | |||||
| Intermediate-1 | 8 | |||||
| Intermediate-2 | 7 | |||||
| High | 0 | |||||
| DIPSS Plus | NA | NA | ||||
| Low | 12 | |||||
| Intermediate-1 | 6 | |||||
| Intermediate-2 | 8 | |||||
| High | 1 | |||||
| Treatment | ||||||
| Hydroxyurea | 18 | 7 | 6 | 5 | 1 | 0 |
| Anagrelide | 20 | 4 | 8 | 4 | 4 | 0 |
| Ruxolitinib | 0 | 9 | 10 | 5 | 4 | 1 |
| Interferon | 4 | 5 | 4 | 2 | 1 | 1 |
| Anticoagulation | 6 | 5 | 6 | 5 | 1 | 0 |
| Antiplatelet | 35 | 26 | 19 | 11 | 7 | 1 |
| Variable . | ET . | PV . | MF . | PMF . | post ET-MF . | post PV-MF . |
|---|---|---|---|---|---|---|
| No. of patients per subgroup | 47 | 30 | 28 | 18 | 9 | 1 |
| Sex | ||||||
| Female | 28 | 14 | 17 | |||
| Male | 19 | 16 | 11 | |||
| Median age, y | 64.6 | 65.3 | 67.7 | 60.6 | 66.1 | 76.5 |
| Median duration of disease, y | 11.5 | 6.2 | 11 | 5.1 | 22.0 | 4.8 |
| Median MPN Symptom Assessment Form total symptom score | 11.0 | 15.5 | 8.0 | |||
| ECOG PS | ||||||
| 0 | 39 | 27 | 24 | |||
| 1 | 4 | 3 | 2 | |||
| 2 | 4 | 0 | 1 | |||
| Mutational status* | ||||||
| JAK2V617F and exon 12 | 31 | 30 | 19 | 12 | 6 | 1 |
| Type I and II CALR | 10 | 0 | 7 | 5 | 2 | 0 |
| MPL | 0 | 0 | 2 | 2 | 0 | 0 |
| Triple negative | 6 | 0 | 1 | 0 | 1 | 0 |
| No. of patients with vascular events | 11 (23.4) | 9 (30) | 8 (28.6) | 5 | 3 | 0 |
| Arterial | 9 | 7 | 7 | 2 | 2 | 0 |
| Venous | 11 | 7 | 7 | 5 | 2 | 0 |
| Splanchnic | 3 | 2 | 2 | 2 | 0 | 0 |
| Bleeding | 1 | 0 | 0 | |||
| Risk scores | ||||||
| IPSET-T | NA | NA | ||||
| Low | 10 | |||||
| Intermediate | 11 | |||||
| High | 26 | |||||
| ELN | NA | |||||
| Low | 10 | 7 | ||||
| High | 37 | 23 | ||||
| IPSET | NA | NA | ||||
| Low | 10 | |||||
| Intermediate | 30 | |||||
| High | 7 | |||||
| IPSS | NA | (unknown 4) | ||||
| Low | 10 | 16 | ||||
| Intermediate-1 | 5 | 5 | ||||
| Intermediate-2 | 11 | 6 | ||||
| High | 0 | |||||
| DIPSS | NA | NA | ||||
| Low | 13 | |||||
| Intermediate-1 | 8 | |||||
| Intermediate-2 | 7 | |||||
| High | 0 | |||||
| DIPSS Plus | NA | NA | ||||
| Low | 12 | |||||
| Intermediate-1 | 6 | |||||
| Intermediate-2 | 8 | |||||
| High | 1 | |||||
| Treatment | ||||||
| Hydroxyurea | 18 | 7 | 6 | 5 | 1 | 0 |
| Anagrelide | 20 | 4 | 8 | 4 | 4 | 0 |
| Ruxolitinib | 0 | 9 | 10 | 5 | 4 | 1 |
| Interferon | 4 | 5 | 4 | 2 | 1 | 1 |
| Anticoagulation | 6 | 5 | 6 | 5 | 1 | 0 |
| Antiplatelet | 35 | 26 | 19 | 11 | 7 | 1 |
All data are n (%) unless otherwise stated. In 2 patients, diagnosis was re-evaluated; because of the change in diagnosis, assays were replicated.
DIPSS, Dynamic International Prognostic Scoring System; DIPSS Plus, a refined DIPSS for primary myelofibrosis that incorporates prognostic information from karyotype, platelet count, and transfusion status; ECOG PS, Eastern Cooperative Oncology Group performance score; ELN, European LeukemiaNet; IPSET, International Prognostic Score of Thrombosis for Essential Thrombocythemia; IPSET-T, IPSET-Thrombosis; IPSS, International Prognostic Scoring System; MF, myelofibrosis;
One patient carried both an MPL and a CALR mutation; 2 patients were analyzed twice.