Prognostic impact of bone marrow fibrosis in polycythemia vera: validation of the IWG-MRT study and additional observations

Blood Cancer J. 2017 Mar 10;7(3):e538. doi: 10.1038/bcj.2017.17.

Abstract

In 2012, the International Working Group for Myeloproliferative Neoplasms (MPN) Research and Treatment (IWG-MRT) reported an associations between mild bone marrow (BM) fibrosis (⩾grade 1) in polycythemia vera (PV) and a lower incidence of thrombosis during the clinical course and a higher risk of fibrotic progression. The objective in the current study of 262 patients with PV was to validate these observations and also identify other risk factors for myelofibrosis-free survival (MFFS). About 127 (48%) patients displayed ⩾grade 1 reticulin fibrosis at the time of diagnosis; presenting clinical and laboratory features were not significantly different between patients with or without BM fibrosis. In univariate analysis, BM fibrosis had no significant impact on overall, leukemia-free or thrombosis-free survival, whereas a significant association was noted for MFFS (P=0.009, hazard ratio 2.9; 95% confidence interval 1.32-6.78); other risk factors for MFFS included leukocytosis ⩾15 × 109/l, presence of palpable splenomegaly and abnormal karyotype. During multivariable analysis, leukocytosis ⩾15 × 109/l, palpable splenomegaly and ⩾grade 1 BM reticulin fibrosis remained significant. The current study validates the previously observed association between ⩾grade 1 BM reticulin fibrosis in PV and subsequent fibrotic progression, and identifies leukocytosis and palpable splenomegaly as additional risk factors for fibrotic progression; additional studies are required to clarify the impact of BM fibrosis on thrombosis and that of abnormal karyotype on MFFS.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Biomarkers
  • Biopsy
  • Bone Marrow / metabolism
  • Bone Marrow / pathology*
  • Disease Progression
  • Female
  • Fibrosis
  • Humans
  • Janus Kinase 2 / genetics
  • Kaplan-Meier Estimate
  • Male
  • Middle Aged
  • Mutation
  • Polycythemia Vera / diagnosis*
  • Polycythemia Vera / genetics
  • Polycythemia Vera / mortality*
  • Primary Myelofibrosis / diagnosis
  • Primary Myelofibrosis / mortality
  • Prognosis
  • Reticulin / metabolism
  • Young Adult

Substances

  • Biomarkers
  • Reticulin
  • Janus Kinase 2