Outcome of Ph negative myeloproliferative neoplasms transforming to accelerated or leukemic phase

Leuk Lymphoma. 2018 Dec;59(12):2812-2820. doi: 10.1080/10428194.2018.1441408. Epub 2018 Apr 4.

Abstract

Myeloproliferative neoplasms (MPN) are chronic disorders that can sometimes evolve into accelerated or leukemic phases. We retrospectively identified 122 patients with such blastic phases. The overall median survival was four months: 10.2 months for patients treated with intensive treatments compared to three months for best supportive care (p = .005). Azacytidine, intensive chemotherapies, or allogeneic stem cell transplantation gave the highest median survivals with 9, 10.2, and 19.4 months, respectively. Accelerated phases (AP) had a longer median survival compared to acute leukemia (4.8 months vs. 3.1 months; p = .02). In this retrospective and observational study, we observe that the longest survivals are seen in patients eligible for intensive treatments. Azacytidine shows interesting results in patients non-fit for intensive chemotherapy. Supportive care should probably be restricted to elderly patients and those with unfavorable karyotype. An early diagnosis of AP could also result in a better survival rate.

Keywords: Myeloproliferative neoplasms; allogeneic stem cell transplantation; blastic phase; hypomethylating agents.

Publication types

  • Comparative Study
  • Multicenter Study
  • Observational Study

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
  • Azacitidine / therapeutic use*
  • Blast Crisis / mortality
  • Blast Crisis / therapy*
  • Cross-Sectional Studies
  • Female
  • Hematopoietic Stem Cell Transplantation*
  • Humans
  • Leukemia, Myeloid, Acute / mortality
  • Leukemia, Myeloid, Acute / therapy*
  • Male
  • Middle Aged
  • Palliative Care / methods
  • Retrospective Studies
  • Survival Analysis
  • Survival Rate
  • Transplantation, Homologous
  • Treatment Outcome

Substances

  • Azacitidine