Characteristics and clinical features of primary myelofibrosis patients with and without pulmonary hypertension.
| With PH (N=6) | Without PH (N=15) | ||
|---|---|---|---|
| Age (yr), median (range) | 77 (69–82) | 68 (36–86) | 0.067 |
| Gender, male:female | 3:3 | 9:6 | 1.000 |
| Stage, N (%) | 0.445 | ||
| Prefibrotic/early | 1 (16.7) | 4 (33.3) | |
| Overt myelofibrosis | 5 (83.3) | 10 (66.6) | |
| IPSS, N (%) | 0.398 | ||
| Low | 0 (0) | 4 (26.7) | |
| Intermediate-1 | 3 (50.0) | 8 (53.3) | |
| Intermediate-2 | 1 (16.7) | 1 (6.7) | |
| High | 2 (33.3) | 2 (13.3) | |
| Laboratory findings, mean±SD | |||
| WBC (×109/L) | 22.0±16.7 | 13.8±8.0 | 0.141 |
| Monocyte (×109/L) | 1.3±0.5 | 0.8±0.4 | 0.031 |
| Hemoglobin (g/dL) | 10.8±1.2 | 11.6±3.5 | 0.590 |
| Platelet (×109/L) | 965.0±615.0 | 586.3±437.8 | 0.126 |
| LDH (×UNL) | 2.6±1.6 | 2.4±1.6 | 0.755 |
| Driver gene mutation, N (%) | |||
| 4/6 (66.7) | 10/13 (76.9) | 0.571 | |
| 2/6 (33.3) | 3/13 (23.1) | 0.811 | |
| Comorbidities, N (%) | |||
| Diabetes mellitus | 1 (16.7) | 4 (26.7) | 0.627 |
| Hypertension | 3 (50.0) | 9 (60.0) | 0.676 |
| Chronic kidney disease | 2 (33.3) | 5 (33.3) | 1.000 |
| Smoking | 1 (16.7) | 2 (13.3) | 0.844 |
| Thrombotic eventsa) | 1 (16.7) | 2 (13.3) | 0.844 |
| Follow-up duration (yr), median (range) | 2.4 (0.4–4.1) | 2.8 (0.2–7.0) | 0.529 |
a)Overall thrombotic events (before, at the time of, and after diagnosis).
Abbreviations: CALR, calreticulin; IPSS, International Prognostic Scoring System; LDH, lactate dehydrogenase; PH, pulmonary hypertension; UNL, upper normal limit.
Blood Res 2020;55:77~84 https://doi.org/10.5045/br.2020.2020001
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