Clinicopathologic features and prognostic indicators in Chinese patients with myelofibrosis

To define the clinicopathologic features, outcome, and prognostic indicators of myelofibrosis (MF) in Asian patients.

Two hundred and seventy consecutive Chinese patients (primary MF, n = 207; post-polycythemia vera MF, n = 27; and post-essential thrombocythemia MF, n = 36) from seven regional referral hospitals were analyzed.

The median overall survival (OS) for primary MF was 66 months. Multivariate analysis showed that age >65 years (P = 0.02), platelet count <100 × 10(9)/l (P = 0.001), and leukemic transformation (P = 0.001) negatively impacted on OS. The median OS of 63 patients with secondary MF was 44 months. In primary MF, the 10-year cumulative risk of leukemic transformation was 28%. On multivariate analysis, unfavorable karyotypes significantly predicted inferior leukemia-free survival (LFS) (P = 0.03). In secondary MF, the 10-year cumulative risk of leukemic transformation was 31%. Circulating blasts ≥1% significantly predicted inferior LFS (P = 0.04). The international prognostic scoring system (IPSS) and dynamic IPSS were not significant survival predictors in our cohort. Eighteen patients underwent allogeneic hematopoietic stem cell transplantation. The median OS post-transplantation was merely 19 months.

Platelet count <100 × 10(9)/l, unfavorable karyotypes, and circulating blasts >1% were negative prognostic indicators. Conclusion Chinese MF patients were similar to Western patients in clinicopathologic features and outcome.