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⁎
- Creators
- Palandri, Francesca
- Palumbo, Giuseppe A
- Iurlo, Alessandra
- Polverelli, Nicola
- Benevolo, Giulia
- Breccia, Massimo
- Abruzzese, Elisabetta
- Tiribelli, Mario
- Bonifacio, Massimiliano
- Tieghi, Alessia
- Isidori, Alessandro
- Martino, Bruno
- Sgherza, Nicola
- D'Adda, Mariella
- Bergamaschi, Micaela
- Crugnola, Monica
- Cavazzini, Francesco
- Bosi, Costanza
- Binotto, Gianni
- Auteri, Giuseppe
- Latagliata, Roberto
- Ibatici, Adalberto
- Scaffidi, Luigi
- Penna, Domenico
- Cattaneo, Daniele
- Soci, Francesco
- Trawinska, Malgorzata
- Russo, Domenico
- Cuneo, Antonio
- Semenzato, Giampietro
- Di Raimondo, Francesco
- Aversa, Franco
- Lemoli, Roberto M
- Heidel, Florian
- Reggiani, Maria L B
- Bartoletti, Daniela
- Cavo, Michele
- Catani, Lucia
- Vianelli, Nicola
- Others:
- Palandri, Francesca
- Palumbo, Giuseppe A
- Iurlo, Alessandra
- Polverelli, Nicola
- Benevolo, Giulia
- Breccia, Massimo
- Abruzzese, Elisabetta
- Tiribelli, Mario
- Bonifacio, Massimiliano
- Tieghi, Alessia
- Isidori, Alessandro
- Martino, Bruno
- Sgherza, Nicola
- D'Adda, Mariella
- Bergamaschi, Micaela
- Crugnola, Monica
- Cavazzini, Francesco
- Bosi, Costanza
- Binotto, Gianni
- Auteri, Giuseppe
- Latagliata, Roberto
- Ibatici, Adalberto
- Scaffidi, Luigi
- Penna, Domenico
- Cattaneo, Daniele
- Soci, Francesco
- Trawinska, Malgorzata
- Russo, Domenico
- Cuneo, Antonio
- Semenzato, Giampietro
- Di Raimondo, Francesco
- Aversa, Franco
- Lemoli, Roberto M
- Heidel, Florian
- Reggiani, Maria L B
- Bartoletti, Daniela
- Cavo, Michele
- Catani, Lucia
- Vianelli, Nicola
Description
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.
Additional details
- URL
- http://hdl.handle.net/11567/935262
- URN
- urn:oai:iris.unige.it:11567/935262
- Origin repository
- UNIGE