Hematology
Contributions
Type: Publication Only
Background
Chronic Myeloproliferative neoplasms (CMPN) are Philadelphia-negative malignancies characterized by a clonal proliferation of one or several lineages. According to WHO classification, CMPNs include Polycythemia Vera (PV), Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF).
Aims
The aim of this report was to determine the demographic features, disease characteristics, JAK mutational status, treatment strategies, and survival rates of 708 patients with CMPN from 8 centers in Turkey.
Methods
Across all of Turkey, 8 centers were enrolled in the study. We retrospectively evaluated 708 patients’ results with CMPN.
Results
The JAK2V617F mutation was found positive in 75.11% of patients with PV, in 51.5% of patients with ET and in 50.4% of patients with PMF. Thrombosis occurred in 20.65% of patients with PV, arterial in 21 (47.7%) cases and venous in 23 (52.2%). Thrombosis was observed in 15.12% of patients with ET, arterial in 30 (50.8%) cases, venous in 27(45.8%) and two (3.4%) patients suffered both arterial and venous thrombosis. Approximately ten percent of PMF patients suffered from thrombosis, arterial in 7 (6.7%) cases and venous in 3 (2.9%). Bleeding at diagnosis occurred in 7.5% of PV patients, in 9% of ET patients and in 10.4% of PMF patients.
Six hundred and eight patients (85.9%) had a cytoreductive therapy. The most common used drug was hydroxyurea (75.1%). Hydroxyurea was used 79.3% of PV patients, 57.1% of PMF patients, and 77.7% of ET patients in the first-line treatment. Interferon was used 5.1% of PV patients, 1.9% of PMF patients, and 5.1% of ET patients. Cytoreductive therapy was changed in 198 (28% of all) patients. In the second-line treatment, the most common used drug was anagrelide (147 of 198 patients). Anti-platelet therapy was used in 553 (78.1%) patients. Splenectomy was performed in 10 (1.4%) patients. Eight PMF patients (1.1% of all CMPN patients) were treated with allogeneic stem cell transplantation. Progression to acute leukemia and secondary myelofibrosis were observed in 0.6 % and 11.3% of all patients, respectively.
The median follow- up was 38 months (0-322) and overall survival (OS) was 86.7% at 10 years in all patients. Among the 213 PV patients, the median follow- up was 3.5 months (0-322) and the OS was 89.7% at 10 years. In 390 ET patients, the median follow- up was 35 months (0-280) and the OS was 98.5% at 10 years. Among the 105 PMF patients, the median follow-up was 15.5 months (0-229) and the OS was 82.5% at 10 years.
Summary
Our patients results is compatible the literature except the frequency of JAK2V617F mutation in PV patients. Hydroxyurea was the most common used cytoreductive therapy in our country.
Keyword(s): Essential Thrombocytemia, Hydroxyurea, Myelofibrosis, Polycythemia vera
Session topic: Publication Only
Type: Publication Only
Background
Chronic Myeloproliferative neoplasms (CMPN) are Philadelphia-negative malignancies characterized by a clonal proliferation of one or several lineages. According to WHO classification, CMPNs include Polycythemia Vera (PV), Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF).
Aims
The aim of this report was to determine the demographic features, disease characteristics, JAK mutational status, treatment strategies, and survival rates of 708 patients with CMPN from 8 centers in Turkey.
Methods
Across all of Turkey, 8 centers were enrolled in the study. We retrospectively evaluated 708 patients’ results with CMPN.
Results
The JAK2V617F mutation was found positive in 75.11% of patients with PV, in 51.5% of patients with ET and in 50.4% of patients with PMF. Thrombosis occurred in 20.65% of patients with PV, arterial in 21 (47.7%) cases and venous in 23 (52.2%). Thrombosis was observed in 15.12% of patients with ET, arterial in 30 (50.8%) cases, venous in 27(45.8%) and two (3.4%) patients suffered both arterial and venous thrombosis. Approximately ten percent of PMF patients suffered from thrombosis, arterial in 7 (6.7%) cases and venous in 3 (2.9%). Bleeding at diagnosis occurred in 7.5% of PV patients, in 9% of ET patients and in 10.4% of PMF patients.
Six hundred and eight patients (85.9%) had a cytoreductive therapy. The most common used drug was hydroxyurea (75.1%). Hydroxyurea was used 79.3% of PV patients, 57.1% of PMF patients, and 77.7% of ET patients in the first-line treatment. Interferon was used 5.1% of PV patients, 1.9% of PMF patients, and 5.1% of ET patients. Cytoreductive therapy was changed in 198 (28% of all) patients. In the second-line treatment, the most common used drug was anagrelide (147 of 198 patients). Anti-platelet therapy was used in 553 (78.1%) patients. Splenectomy was performed in 10 (1.4%) patients. Eight PMF patients (1.1% of all CMPN patients) were treated with allogeneic stem cell transplantation. Progression to acute leukemia and secondary myelofibrosis were observed in 0.6 % and 11.3% of all patients, respectively.
The median follow- up was 38 months (0-322) and overall survival (OS) was 86.7% at 10 years in all patients. Among the 213 PV patients, the median follow- up was 3.5 months (0-322) and the OS was 89.7% at 10 years. In 390 ET patients, the median follow- up was 35 months (0-280) and the OS was 98.5% at 10 years. Among the 105 PMF patients, the median follow-up was 15.5 months (0-229) and the OS was 82.5% at 10 years.
Summary
Our patients results is compatible the literature except the frequency of JAK2V617F mutation in PV patients. Hydroxyurea was the most common used cytoreductive therapy in our country.
Keyword(s): Essential Thrombocytemia, Hydroxyurea, Myelofibrosis, Polycythemia vera
Session topic: Publication Only