Table 1 Patient characteristics, for the entire cohort, for ET and PV patients.
ALL (n = 348) | ET (n = 278) | PV (n = 70) | p-value | ||||
---|---|---|---|---|---|---|---|
n | % | n | % | n | % | ||
Age at diagnosis (median, IQR/range) | 20yo | 18–23/2–25 | 21yo | 18–23/2–25 | 20yo | 16–23/2–25 | 0.320 |
Sex | <0.001 | ||||||
Male | 99 | 28.4 | 62 | 22.3 | 37 | 52.9 | |
Female | 249 | 71.6 | 216 | 77.7 | 33 | 47.1 | |
Mutational status | <0.001 | ||||||
JAK2 | 217 | 62.4 | 147 | 52.9 | 70 | 100.0 | |
Exon 14 | 212 | 147 | 65 | ||||
Allele burden (median, IQR) (data available for 58 pts) | 22.4 | 14–22 | 15.8 | 10.7–25.5 | 32.5 | 22.5–44 | <0.001 |
Exon 12 | 5 | 0 | 5 | ||||
CALR | 43 | 12.3 | 43 | 15.5 | 0 | 0.0 | |
Type 1 | 14 | 14 | 0 | ||||
Type 2 | 7 | 7 | 0 | ||||
Unknown | 22 | 22 | 0 | ||||
MPL | 3 | 0.9 | 3 | 1.1 | 0 | 0.0 | |
TN | 85 | 24.4 | 85 | 30.5 | 0 | 0.0 | |
Biochemical analysis | |||||||
Hemoglobin (g/L) (median, IQR) (data available for 288 pts) | 141.5 | 131–153 | 139 | 130–143 | 170 | 134–240 | <0.001 |
Hematocrit (%) (median, IQR) (data available for 269 pts) | 42.9 | 39.3–45.7 | 41.5 | 39–44 | 52 | 48.3–57.4 | <0.001 |
Hematocrit >45% (data available for 269 pts) | 84 | 31.2 | 41 | 18.4 | 43 | 93.5 | <0.001 |
White blood cell (×109/ul) (median, IQR) (data available for 288 pts) | 9 | 7.5–11.2 | 8.8 | 7.4–10.7 | 11.4 | 7.1–13.3 | 0.001 |
White blood cell >11 ×109/ul (data available for 288 pts) | 77 | 26.7 | 52 | 21.5 | 25 | 54.3 | <0.001 |
Platelets (×109/ul) (median, IQR) (data available for 293 pts) | 874 | 621–1253 | 913 | 664–1304 | 628 | 355–919 | <0.001 |
Platelet count >1000 ×109/ul (data available for 293 pts) | 106 | 36.2 | 99 | 40.4 | 7 | 14.6 | <0.001 |
LDH above normal range (data available for 217 pts) | 98 | 45.2 | 73 | 42 | 25 | 58.1 | 0.056 |
Splenomegaly by palpation (data available for 305 pts) | 54 | 17.7 | 33 | 13.1 | 21 | 38.9 | <0.001 |
Reason for consulting | <0.001 | ||||||
Thrombotic event | 27 | 7.8 | 13 | 4.7 | 14 | 20.0 | |
Hemorrhage | 0 | 0.0 | 0 | 0.0 | 0 | 0.0 | |
MPN-related symptoms | 72 | 20.7 | 57 | 20.5 | 15 | 21.4 | |
Abnormal blood count | 71 | 20.4 | 66 | 23.7 | 5 | 7.2 | |
Abnormal blood count during pregnancy | 15 | 4.3 | 12 | 4.3 | 3 | 4.3 | |
Unknown | 163 | 46.8 | 130 | 46.8 | 33 | 47.1 | |
MPN-related symptoms | |||||||
Plethoric face (data available for 296 pts) | 10 | 3.4 | 0 | 0.0 | 10 | 19.6 | <0.001 |
Pruritus (data available for 304 pts) | 16 | 5.3 | 7 | 2.8 | 9 | 16.1 | <0.001 |
Hyperviscosity (data available for 296 pts) | 101 | 35.3 | 78 | 33.8 | 23 | 41.8 | 0.261 |
Constitutive symptoms (data available for 299 pts) | 14 | 4.0 | 10 | 3.6 | 4 | 5.7 | 0.088 |
Fatigue (data available for 281 pts) | 55 | 19.6 | 38 | 16.3 | 17 | 35.4 | 0.002 |
Sweets (data available for 281 pts) | 5 | 1.8 | 1 | 0.4 | 4 | 8.3 | <0.001 |
Vascular symptoms (data available for 318 pts) | 34 | 10.7 | 30 | 11.6 | 4 | 6.7 | 0.263 |
CVRF (presence of any) (data available for 341 pts) | 46 | 13.5 | 35 | 12.8 | 11 | 16.2 | 0.469 |
Smoking | 32 | 9.4 | 26 | 9.5 | 6 | 8.8 | 0.859 |
High blood pressure | 7 | 2.1 | 4 | 1.5 | 3 | 4.4 | 0.145 |
Dyslipidemia | 1 | 0.3 | 1 | 0.4 | 0 | 0.0 | 1.000 |
Diabetes | 2 | 0.6 | 0 | 0.0 | 2 | 2.9 | 0.039 |
Obesity | 5 | 1.5 | 4 | 1.5 | 1 | 1.5 | 1.000 |
Thrombosis history (data available for 347 pts) | 43 | 12.4 | 26 | 9.4 | 17 | 24.6 | <0.001 |
Venous | 31 | 8.9 | 21 | 7.6 | 10 | 14.5 | 0.070 |
BCS | 11 | 3.2 | 6 | 2.2 | 5 | 7.2 | |
BCS/CVT | 1 | 0.3 | 0 | 0.0 | 1 | 1.4 | |
CVT | 10 | 2.9 | 7 | 2.5 | 3 | 4.3 | |
DVT | 3 | 0.9 | 3 | 1.1 | 0 | 0.0 | |
PE | 2 | 0.6 | 2 | 0.7 | 0 | 0.0 | |
PVT | 2 | 0.6 | 1 | 0.4 | 1 | 1.4 | |
RVT | 1 | 0.3 | 1 | 0.4 | 0 | 0.0 | |
SpVT | 1 | 0.3 | 1 | 0.4 | 0 | 0.0 | |
Arterial | 9 | 2.6 | 3 | 1.1 | 6 | 8.7 | 0.003 |
AMI | 3 | 0.9 | 1 | 0.4 | 2 | 2.9 | |
Stroke | 2 | 0.6 | 1 | 0.4 | 1 | 1.4 | |
TIA | 4 | 1.2 | 1 | 0.4 | 3 | 4.3 | |
Unknown localisation | 3 | 0.9 | 2 | 0.7 | 1 | 1.4 | |
Bleeding history (data available for 347 pts) | 20 | 5.8 | 17 | 6.1 | 3 | 4.3 | 0.775 |
ELN risk | <0.001 | ||||||
Low | 304 | 87.6 | 252 | 90.6 | 52 | 75.4 | |
High | 43 | 12.4 | 26 | 9.4 | 17 | 24.6 | |
IPSET thrombosis (data available for 269 pts) | |||||||
Low | N/A | 122 | 45.4 | N/A | |||
Intermediate | N/A | 110 | 40.9 | N/A | |||
High | N/A | 37 | 13.7 | N/A |