Nationwide prospective survey of secondary myelofibrosis in Japan: superiority of DIPSS-plus to MYSEC-PM as a survival risk model
Kotaro Shide, Katsuto Takenaka, Akira Kitanaka, Akihiko Numata, Takuro Kameda, Takuji Yamauchi, Atsushi Inagaki, Shohei Mizuno, Akiyoshi Takami, Shinichi Ito, Masao Hagihara, Kensuke Usuki, Takaaki Maekawa, Kazutaka Sunami, Yasunori Ueda, Miyuki Tsutsui, Miki Ando, Norio Komatsu, Keiya Ozawa, Mineo Kurokawa, Shunya Arai, Kinuko Mitani, Koichi Akashi, Kazuya Shimoda
Abstract
Myelofibrosis (MF) is divided into two categories, namely primary MF (PMF) and secondary MF arising from essential thrombocythemia (ET) or polycythemia vera (PV), so-called PET-MF or PPV-MF, respectively [ 1 ]. ET and PV are considered dormant MPNs, and their progression to MF is a natural evolution. 15 years-cumulative incidence of PET-MF and PPV-MF were 4–11% and 6–14%, respectively [ 2 ]. Patients with PET/PPV-MF and PMF are similarly managed in clinical practice [ 3 ]. However, discrepancies in the application of PMF prognostic scoring systems, such as the International Prognostic Scoring System (IPSS) and dynamic IPSS (DIPSS) for patients with PET/PPV-MF have been reported [ 4 , 5 , 6 , 7 , 8 ], and the prognostic scoring systems for PET/PPV-MF has been developed [ 9 ]. We evaluated the existing prognostic models developed for PMF and PET/PPV-MF in a nationwide prospective observational study for patients with PET/PPV-MF conducted by the Japanese National Research Group on Idiopathic Bone Marrow Failure Syndromes.