Literature DB >> 29616837

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

Lise-Marie Mollard1, Aurélie Chauveau2, Françoise Boyer-Perrard3, Nathalie Douet-Guilbert4, Roch Houot5, Isabelle Quintin-Roué6, Marie-Anne Couturier7, Anaig Dagorne8, Mohamed Malou9, Ronan Le Calloch10, Odile Luycx11, Sylvain Thepot3, Mathilde Hunault3, Gaelle Guillerm1, Christian Berthou1, Valérie Ugo9, Éric Lippert2, Jean-Christophe Ianotto1.   

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.

Entities:  

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

Mesh:

Substances:

Year:  2018        PMID: 29616837     DOI: 10.1080/10428194.2018.1441408

Source DB:  PubMed          Journal:  Leuk Lymphoma        ISSN: 1026-8022


  3 in total

1.  Advanced forms of MPNs are accompanied by chromosomal abnormalities that lead to dysregulation of TP53.

Authors:  Bridget K Marcellino; Ronald Hoffman; Joseph Tripodi; Min Lu; Heidi Kosiorek; John Mascarenhas; Raajit K Rampal; Amylou Dueck; Vesna Najfeld
Journal:  Blood Adv       Date:  2018-12-26

2.  Leukemic evolution of polycythemia vera and essential thrombocythemia: genomic profiles predict time to transformation.

Authors:  Damien Luque Paz; Rébecca Jouanneau-Courville; Jérémie Riou; Jean-Christophe Ianotto; Françoise Boyer; Aurélie Chauveau; Maxime Renard; Jean-Claude Chomel; Emilie Cayssials; Maria-Pilar Gallego-Hernanz; Cédric Pastoret; Anne Murati; Frédéric Courtier; Marie-Christine Rousselet; Isabelle Quintin-Roué; Laurane Cottin; Corentin Orvain; Sylvain Thépot; Jean-Marie Chrétien; Yves Delneste; Norbert Ifrah; Odile Blanchet; Mathilde Hunault-Berger; Eric Lippert; Valérie Ugo
Journal:  Blood Adv       Date:  2020-10-13

Review 3.  Accelerated Phase of Myeloproliferative Neoplasms.

Authors:  Omar A Shahin; Helen T Chifotides; Prithviraj Bose; Lucia Masarova; Srdan Verstovsek
Journal:  Acta Haematol       Date:  2021-04-21       Impact factor: 2.195

  3 in total

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