Differences in presenting features, outcome and prognostic models in patients with primary myelofibrosis and post-polycythemia vera and/or post-essential thrombocythemia myelofibrosis treated with ruxolitinib. New perspective of the MYSEC-PM in a large multicenter study

Francesca Palandri, Giuseppe A. Palumbo, Alessandra Iurlo, Nicola Polverelli, Giulia Benevolo, Massimo Breccia, Elisabetta Abruzzese, Mario Tiribelli, Massimiliano Bonifacio, Alessia Tieghi, Alessandro Isidori, Bruno Martino, Nicola Sgherza, Mariella D'Adda, Micaela Bergamaschi, Monica Crugnola, Francesco Cavazzini, Costanza Bosi, Gianni Binotto, Giuseppe AuteriRoberto Latagliata, Adalberto Ibatici, Luigi Scaffidi, Domenico Penna, Daniele Cattaneo, Francesco Soci, Malgorzata Trawinska, Domenico Russo, Antonio Cuneo, Giampietro Semenzato, Francesco Di Raimondo, Franco Aversa, Roberto M. Lemoli, Florian Heidel, Maria L.B. Reggiani, Daniela Bartoletti, Michele Cavo, Lucia Catani, Nicola Vianelli

Research output: Contribution to journalArticle

Abstract

Recently, the myelofibrosis secondary to PV and ET prognostic model (MYSEC-PM) was introduced to assess prognosis in myelofibrosis (MF) secondary to polycythemia vera and essential thrombocythemia (post-PV and post-ET MF), replacing the International Prognostic Scoring System (IPSS) and/or Dynamic IPSS (DIPSS) that was applied for primary MF (PMF). In a cohort of 421 ruxolitinib (RUX)-treated patients (post-PV and post-ET MF: 44.2%), we evaluated the following: (1) disease phenotype, responses, and toxicity to RUX; and (2) performance of the MYSEC-PM in post-PV or post-ET MF. While the IPSS failed to correctly stratify post-PV or post-ET MF patients at diagnosis, the MYSEC-PM identified 4 risk categories projected at significantly different survival probability (P <.001). Additionally, the MYSEC-PM maintained a prognostic value in post-PV and post-ET MF also when used over time, at RUX start. Notably, the MYSEC-PM reclassified 41.8% and 13.6% of patients into a lower and higher risk category, respectively. Finally, patients at intermediate-1 risk had significantly higher spleen responses and lower hematological toxicities compared to higher risk patients. Compared to PMF, post-PV and post-ET MF presented a more hyperproliferative disease, with higher leukocyte and/or platelet count and hemoglobin levels both at diagnosis and at RUX start. Despite comparable response rates, post-PV and post-ET MF had lower rate of RUX-induced anemia and thrombocytopenia at 3 and 6 months. The study validates MYSEC-PM in post-PV and post-ET MF prognostication. Post-PV or post-ET MF represents a separate entity compared to PMF in terms of clinical manifestations and toxicity to RUX.

Original languageEnglish
Pages (from-to)248-255
JournalSeminars in Hematology
Volume55
Issue number4
DOIs
Publication statusPublished - 2018

Keywords

  • IPSS
  • Myelofibrosis
  • MYSEC-PM
  • Risk scores
  • Ruxolitinib

ASJC Scopus subject areas

  • Hematology

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    Palandri, F., Palumbo, G. A., Iurlo, A., Polverelli, N., Benevolo, G., Breccia, M., Abruzzese, E., Tiribelli, M., Bonifacio, M., Tieghi, A., Isidori, A., Martino, B., Sgherza, N., D'Adda, M., Bergamaschi, M., Crugnola, M., Cavazzini, F., Bosi, C., Binotto, G., ... Vianelli, N. (2018). Differences in presenting features, outcome and prognostic models in patients with primary myelofibrosis and post-polycythemia vera and/or post-essential thrombocythemia myelofibrosis treated with ruxolitinib. New perspective of the MYSEC-PM in a large multicenter study. Seminars in Hematology, 55(4), 248-255. https://doi.org/10.1053/j.seminhematol.2018.05.013