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Myelofibrosis: Prognostication and cytoreductive treatment
Margherita Maffioli, Domenica Caramazza, Barbara Mora, Michele Merli, Francesco Passamonti, Division of Hematology, Department of Medicine, University Hospital Ospedale di Circolo e Fondazione Macchi, 21100 Varese, Italy
Author contributions: Maffioli M and Passamonti F wrote the paper; Maffioli M, Caramazza D, Mora B, Merli M and Passamonti F discussed the mail topics of the paper and all gave the final approval of the manuscript.
Correspondence to: Francesco Passamonti, MD, Division of Hematology, Department of Medicine, University Hospital Ospedale di Circolo e Fondazione Macchi, Viale L. Borri 57, 21100 Varese, Italy. francesco.passamonti@ospedale.varese.it
Telephone: +39-332-393648 Fax: +39-332-393648
Received: November 2, 2013
Revised: February 8, 2014
Accepted: June 10, 2014
Published online: August 6, 2014
Processing time: 351 Days and 18.1 Hours
Revised: February 8, 2014
Accepted: June 10, 2014
Published online: August 6, 2014
Processing time: 351 Days and 18.1 Hours
Core Tip
Core tip: Myelofibrosis (MF) is a mutational/clinical-complex disease. Prognostication of MF is based on the International Prognostic scoring system (IPSS) model at diagnosis and on the Dynamic IPSS thereafter. Factors included in both models are: age > 65 years, constitutional symptoms, hemoglobin < 10 g/dL, leukocytes > 25 × 109/L, and circulating blast cells 1% or greater. Cytogenetic profile and mutational status help to better discriminate within each IPSS category. JAK inhibitors are new promising therapies with a molecular target, translating into a clinical benefit: spleen reduction MF-symptoms refief. Among JAK inhibitor, ruxolitinib has been approved for MF.