Table 2 Studies evaluating outcomes in polycythemia vera

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

  Kiladjian et al.7 Passamonti et al.11 Finazzi et al.12 Marchioli et al.13 Gangat et al.14 Passamonti et al.15 Abdulkarim et al.16 Tefferi et al.3 Bonicelli et al.17 Stein et al.18 Sever et al.19 Tefferi et al.2 Bai et al.20
N 164 396 1638 1638 459 320 150 1545 327 204 133 267 (Mayo)
310 (Italy)
272
Median F/U (years) 11.4 9.6 2.8 2.7 5.3 3.2 15 6.9 11 8 (age 45 years)
4.5 (age 65 years)
7.5 11.8
11.1
6
MF
N 14 (8.5%) 5.1 (3.3–7.8)a NR 38 (2.3%) 54 (12%) 8 (2.5%) 13 (8.5%) 138 (9%) 37 (11%) 26 (12.7%) 11 (8%) 34 (12.7%)
65 (21%)
63 (23%)b
 Median time to MF from Dx (years): 12.5 13 NR NR 10.5 NR NR NR 9.6 20 (age 45 years)
8 (age 65 years)
8.5c 9.6 NR
 Risk factors None Sequential use of 2 myelosuppressive agents compared with Pipobroman NR Disease duration >10 years Age 60 years JAK2V617F allele burden 50% Splenomegaly
Reticulin grading
NR None Median age at MF Dx None None Splenomegaly
Plt>550 × 109/l
JAK2V617F allele burden 50%
Leukemia
N 32 (19.5%) 5.3 (3.5-8)a 22 (1.3%) 21 (1.3%) 34 (7.4%) 10 (3%) 13 (8.5%) 50 (3%) 30 (9.2%) 7 (3%) 4 (3%) 18 (6.7%)
10 (3.2%)
6.6 (3.7-10.8)d
 Median time to LT from Dx (years): 9.6 14 8.4 NR 10.5 NR NR 10.8 4.6 19 (age 45 years)
7 (age 65 years)
8.5c NR NR
 Risk factors WBC10 × 109/l Sequential use of 2 myelosuppressive agents compared with HU or Pipobroman Age 70 years
P32
Busulphan
Pipobroman
Cytoreductive drug alone or in combo
Low cholesterol (150 mg/dl)
Age 70 years
Cytoreductive agents other than HU or IFN
WBC10 × 109/l
WBC15 × 109/l
None Splenomegaly
Reticulin grading
Age >61 years
WBC 15 × 109/l
Abnormal karyotype
P32/CMB alone
Pipobroman
Female None None None Plt<100 × 109/l
Median Survival (years): 15.5 20 NR NRe 22.7 NR NRf 18.9g 17.5 (age <65 years)
6.4 (age 65 years)h
NR 24 13.5i NRj
Risk factors Age 60 years
WBC10 × 109/l
Thrombosis NR Age >65 years
Thrombosis
Age 60 years
WBC15 × 109/l
Arterial thrombosis
Age 60 years Splenomegaly Age >61 years
WBC15 × 109/l Thrombosis
Abnormal karyotype
Pruritusk
Age >70 years
WBC>13 × 109/l
Thrombosis
NR None Age <60 years Age >65 years
WBC>25 × 109/l
Thrombosis
  1. Abbreviations: CMB, chlorambucil; Dx, diagnosis; HU, hydroxyurea; IFN, interferon; LT, leukemic transformation; MF, myelofibrosis; NR, not reported; WBC, white blood cell.
  2. aIncidence per 1000 person-years (95% CI).
  3. bIncidence of 31 (95% CI=24.12–40.16) per 1000 person/year.
  4. cMedian time to leukemic or myelofibrotic transformation.
  5. dIncidence rate per 1000 person/year with 19% leukemic transformation from post-PV MF population (n=63).
  6. eOverall mortality rate of 3.7 per 100 persons per year.
  7. fMedian overall survival 40% at 10 years.
  8. gMedian overall survival 14.1 years based on most mature follow-up cohort (n=337).
  9. hMedian overall survival 72% at 10 years.
  10. iBased on Mayo cohort.
  11. j83% median overall survival at 10 years.
  12. kFavorable risk factor.