Incidence and risk factors for myelofibrotic transformation among 272 Chinese patients with JAK2‐mutated polycythemia vera

Abstract

Post-polycythemia vera myelofibrosis (post-PV MF) is a critical hematologic evolution of polycythemia vera (PV). The main purpose of the present study was to identify the possible risk factors for the occurrence and prognosis of post-PV MF in Chinese patients with PV. A cohort of 272 Chinese PV patients with JAK2 V617F or exon12 mutation was retrospectively analyzed. Of the 272 patients with PV, 63 developed post-PV MF. Platelet count >550 3 10 9 /L and splenomegaly were identified as independent risk factors for post-PV MF. The median duration of survival for post-PV MF patients was 8 years. Anemia and age >65 years at diagnosis of post-PV MF were identified as significant predictors for the poor prognosis of post-PV MF. In conclusion, platelet counts and splenomegaly were significant predictors for the transformation to post-PV MF, while anemia (hemoglobin levels <100 g/L) and age>65 years were significant predictors for poor prognosis of post-PV MF in Chinese PV patients with JAK2 V617F or exon12 mutation.

Thrombosis among 1537 patients with JAK2V617F‐mutated myeloproliferative neoplasms: Risk factors and development of a predictive model

“…MPNs mainly include polycythemia vera (PV), essential thrombocythemia (ET), and primary myelofibrosis (PMF) . Thrombosis, leukemic transformation, and myelofibrotic transformation are critical complications of MPN and are highly common in patients with MPN . Fatalities due to thrombosis account for 35%~70% of the total mortality rate of MPN, making it the most important risk factor affecting event‐free survival and overall survival (OS) of MPN patients …”

JAK2 V617F as a Marker for Long-Term Disease Progression and Mortality in Polycythemia Vera and its Role in Economic Modeling

“…When we combined risk levels to establish a range of possible OS levels, our model provided an OS range of 14.2 to 17.3 LY depending on age and baseline JAK2. Only the study by Bai et al 19 had an OS (17.5 LY) that slightly fell outside of the modeled OS range.…”

“…The results of the validation indicated a good correspondence between the model and the published observational studies available for comparisons. The mean OS in the published observational studies 19 , 31 , 32 that was included in the validation of the model was 14.9 to 17.5 years over a 20-year time horizon. When we adjusted our model’s baseline age and baseline JAK2 burden to match the studies’ variables, our model predicted a mean OS that ranged between 15.0 to 16.5 years.…”

Is there a gender effect in polycythemia vera?

“…To date, sex is not included among risk factors for PV evolution into PPV-MF and BP. Conventional risk factors for PPV-MF and BP transformation include age ≥ 60 years, leukocytosis (≥ 15-30 × 10 9 /L), homozygosity for JAK2 mutation, and exposures to alkylating agents (reviewed by Cuthbert D et al) [103][104][105][106][107][108][109][110]. NGS based studies demonstrated also that non-driver mutations in myeloid genes could influence PV evolution: ASXL1, SRSF2, RUNX1, SF3B1, and IDH1/2 for PPV-MF; ASXL1, TP53, SRSF2, IDH1/2, and RUNX1 for BP [110].…”