Table 3 Studies evaluating outcomes in essential thrombocythemia

From: Blast transformation and fibrotic progression in polycythemia vera and essential thrombocythemia: a literature review of incidence and risk factors

  Passamonti et al.11 Chim et al.29 Wolanskyj et al.28 Gangat et al.37 Passamonti et al.30 Palandri et al.31 Girodon et al.35 Abdulkarim et al.16 Barbui et al.34 Malak et al.32 Tefferi et al.2
N 435 231 322 605 605 386 311 130 891 105 292 (Mayo) 284 (Italy)
Median F/U (years) 9.3 NR 13.6 7 5.6 9.5 9.5 15 6.2 7.5 17.3 10.7
MF
N 1.6 (0.8–3.4)a 7 (3%) NRb NR 17 (2.8%) 20 (5%) NR 7 (5%) 32 (4%) 12 (13%) 29 (9.9%) 26 (9.2%)
 Median time to MF from Dx (years) 10.9 8 12.4 NR 9.1 7.25 NR NR NR 16 NR
 Risk factors NR NR None NR Anemiac Sequential use of cytotoxic drugs NR Anemia Leukocytosis Reticulin
BM cellularity
BM histology
Age >60 years
Hgb<120 g/l
Absent JAK2V617F
None MPL mutated
Leukemia
N 1.2 (0.5–2.8)a 4 (1.7%) NRd 20 (3.3%) 14 (2.3%) 6 (1.5%) 18 (5.8%) 11 (8%) 8 (1%) 7 (10%) 12 (4.1%) 4 (1.4%)
 Median time to LT from Dx (years) 14.5 10 13.8 11.5 11 8.5 6.3 NR NR 7.4 NR
 Risk factors None Previous MF
Melphalan
WBC>11 × 109/l
None Anemiae
Plts 1000 × 109/l
Age (continuous variable)
Age >60 years Sequential use of cytotoxic drugs None Anemia
Leukocytosis Reticulin
BM cellularity
BM histology
Thrombosis
Plts>1000 × 109/l
None None
Median survival (years) 22.6 13 18.9 18 22.3 NR 11 NRf 14.7 NRg 19.8h
Risk factors Thrombosis
Male
Age >60 years Age 60 years
WBC15 × 109/l
Tobacco use
DM
Age 60 years
WBC15 × 109/l
Anemiae
Thrombosis
DM
Smoking
Age >60 years
Thrombosis
Age 60 years
WBC15 × 109/l
HTN
DM
Thrombosis
Age >60 years
WBC>11 × 109/l
Anemia
Anemia Age >60 years
WBC>11 × 109/l
Hgb <120 g/l
Thrombosis
BM histology
NR Age 60 years
  1. Abbreviations: BM, bone marrow; DM, diabetes mellitus; Dx, diagnosis; HTN, hypertension; HU, hydroxyurea; LT, leukemic transformation; MF, myelofibrosis; NR, not reported; Plts, platelets; WBC, white blood cell.
  2. aIncidence per 1000 person-years (95% CI).
  3. b3.8% cumulative probability of MF transformation at 10 years (95% CI=1.4–6.1).
  4. cHgb<125 g/l in females, <135 g/l in males.
  5. d1.4% 10-year cumulative probability of AML transformation (95% CI=0–3).
  6. eHgb<120 g/l in females, <135 g/l in males.
  7. fOverall survival 58% at 10 years.
  8. gOverall survival 83% at 10 years.
  9. hBased on Mayo cohort.