Turk J Haematol 2016 Apr 18
Multicenter-Retrospective Analysis Of Turkish Patients With Chronic Myeloproliferative Neoplasms.
Soyer N, Haznedaroğlu İC, Cömert M, Çekdemir D, Yılmaz M, Ünal A, Çağlıyan G, Bilgir O, İlhan O, Özdemirkıran F, Kaya E, Şahin F, Vural F, Saydam G.
Chronic Myeloproliferative neoplasms (CMPN) that include Polycythemia Vera (PV), Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF) are Philadelphia-negative malignancies characterized by a clonal proliferation of one or several lineages. The aim of this report was to determine the demographic features, disease characteristics, treatment strategies, and survival rates of patients with CMPN in Turkey. Methods: Across all of Turkey, 9 centers were enrolled in the study. We retrospectively evaluated 708 CMPN patients' results including 390 with ET, 213 with PV and 105 with PMF. Results: The JAK2V617F mutation was found positive in 86% of patients with PV, in 51.5% of patients with ET and in 50.4% of patients with PMF. Thrombosis and bleeding at diagnosis occurred in 20.6% and 7.5% of PV patients, 15.1% and 9% of ET patients and, 9.5% and 10.4% of PMF patients, respectively. Six hundred and eight patients (85.9%) had a cytoreductive therapy. The most common used drug was hydroxyurea (89.6%). Leukemic and fibrotic transformations were 0.6% and 13.2%. The estimated OS in PV, ET and PMF patients was 89.7%, 85% and 82.5% at 10 years, respectively. There were no significant difference between survivals in ET, PV and PMF patients at 10 years. Conclusion: Our patients' results are generally compatible with the literature except relatively high survival rate in PMF patients. Hydroxyurea was the most common used cytoreductive therapy in our country. Our study was reflected demographic features, patient's characteristics, treatments, and survival rates of Turkish CMPNs patients.
l="OBJECTIVES" NlmCategory="OBJECTIVE">Chronic Myeloproliferative neoplasms (CMPN) that include Polycythemia Vera (PV), Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF) are Philadelphia-negative malignancies characterized by a clonal proliferation of one or several lineages. The aim of this report was to determine the demographic features, disease characteristics, treatment strategies, and survival rates of patients with CMPN in Turkey. Methods: Across all of Turkey, 9 centers were enrolled in the study. We retrospectively evaluated 708 CMPN patients' results including 390 with ET, 213 with PV and 105 with PMF. Results: The JAK2V617F mutation was found positive in 86% of patients with PV, in 51.5% of patients with ET and in 50.4% of patients with PMF. Thrombosis and bleeding at diagnosis occurred in 20.6% and 7.5% of PV patients, 15.1% and 9% of ET patients and, 9.5% and 10.4% of PMF patients, respectively. Six hundred and eight patients (85.9%) had a cytoreductive therapy. The most common used drug was hydroxyurea (89.6%). Leukemic and fibrotic transformations were 0.6% and 13.2%. The estimated OS in PV, ET and PMF patients was 89.7%, 85% and 82.5% at 10 years, respectively. There were no significant difference between survivals in ET, PV and PMF patients at 10 years. Conclusion: Our patients' results are generally compatible with the literature except relatively high survival rate in PMF patients. Hydroxyurea was the most common used cytoreductive therapy in our country. Our study was reflected demographic features, patient's characteristics, treatments, and survival rates of Turkish CMPNs patients.