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Original Article: Clinical

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

, , , , , , , , , , , , , , , , & show all
Pages 2812-2820 | Received 12 Jun 2017, Accepted 04 Feb 2018, Published online: 04 Apr 2018
 

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.

Acknowledgements

The authors would like to thank Delphine Le Terrec for her precious secretarial help. The authors would like to thank the France Intergroup of Myeloproliferative neoplasms (FIM) for its help throughout this study: AC, FBP, AD, RLC, VU, EL and JCI are members of this cooperative group.

Potential conflict of interest

Disclosure forms provided by the authors are available with the full text of this article online at https://doi.org/10.1080/10428194.2018.1441408.

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