Impact of 2016 WHO diagnosis of early and overt primary myelofibrosis on presentation and outcome of 232 patients treated with ruxolitinib

Hematol Oncol. 2019 Oct;37(4):418-423. doi: 10.1002/hon.2619. Epub 2019 Jun 7.

Abstract

The 2016 WHO criteria identified early primary myelofibrosis (PMF) as an individual entity with milder clinical features and better outcome compared with overt PMF. Here, we compared early and overt PMF patients treated with ruxolitinib in terms of baseline clinical/laboratory characteristics, response, and toxicity to treatment. We observed that early-PMF patients achieve better and more stable spleen and symptoms responses, with significantly lower rates of hematological toxicities. No differences in overall and leukemia-free survival were detected between the two cohorts. The application of 2016 WHO criteria is crucial to identify those PMF patients who deserve a stricter monitoring during treatment.

Keywords: EARLY PMF; MYELOFIBROSIS; OVERT PMF; RUXOLITINIB.

Publication types

  • Comparative Study

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Antineoplastic Agents / therapeutic use*
  • Clinical Laboratory Techniques / standards
  • Diagnosis, Differential
  • Early Diagnosis
  • Female
  • Humans
  • Janus Kinase 2 / antagonists & inhibitors
  • Janus Kinase 2 / genetics
  • Male
  • Middle Aged
  • Molecular Targeted Therapy
  • Neoplasm Proteins / antagonists & inhibitors
  • Neoplasm Proteins / genetics
  • Nitriles
  • Organ Size
  • Primary Myelofibrosis / diagnosis
  • Primary Myelofibrosis / drug therapy*
  • Protein Kinase Inhibitors / therapeutic use*
  • Pyrazoles / therapeutic use*
  • Pyrimidines
  • Retrospective Studies
  • Spleen / pathology
  • Thrombocythemia, Essential / diagnosis
  • Treatment Outcome
  • World Health Organization

Substances

  • Antineoplastic Agents
  • Neoplasm Proteins
  • Nitriles
  • Protein Kinase Inhibitors
  • Pyrazoles
  • Pyrimidines
  • ruxolitinib
  • JAK2 protein, human
  • Janus Kinase 2