Ph Negative Myeloproliferative Neoplasms at Czech Hematological Centers – MIND Analysis
Keywords:
Ph negative myeloproliferative neoplasms – primary myelofibrosis – polycythemia vera – essential thrombocythemia – MIND database – epidemiology – diagnostic – treatment – prognosisAbstract
Background: Our MIND database was initiated on May 1, 2013, with the primary objective of collecting data from patients with Ph negative myeloproliferative neoplasms (Ph-MPN) at Czech hematological centers. Principal research focus was to analyze and compare our data with published data.
Patients and Methods: A total of 641 Ph-MPN patients registered with MIND and were analyzed from 2013 to 2020.
Results: The most common diagnosis was polycythemia vera, PV (34%), primary myelofibrosis, PMF (31%), and essential thrombocythemia, ET (22%). At the time of diagnosis, patients suffered mostly from fatigue (52%) and night sweats (32%). Splenomegaly occurred in more than half of PMF patients (54%). JAK2 V617F mutation was present among 90% of PV, 65% of PMF, and 62% of ET, respectively. Mutation CALR was found in 22% of ET and 16% of PMF, respectively. Cytogenetic abnormalities were significantly documented more frequently in both PMF and PV compared to ET (17% and 15% vs. 3% of examined patients, respectively; p≤0.005). Thrombosis (22%) and bleeding (10%) were the most common complications during diagnosis without any significant difference in frequency among PV, ET, and PMF, respectively (p>0,05). Regarding PV, hydroxyurea was the most frequently used drug during both the 1st and 2nd lines of therapy (72% and 65%, respectively), with anagrelide for both lines in ET (52% and 82%, respectively), hydroxyurea for the 1st line, and ruxolitinib for the 2nd line in PMF (70% and 30%, respectively). 17% of our patients died, mostly from PMF (53%). Median overall survival was not attained in ET, contrasting the lowest median in secondary MF (2,5 years).
Conclusion: We’ve recognized a recurring definitive frequency of constitutional symptoms. A higher incidence of additional cytogenetic abnormalities was recorded more frequently with both PMF and PV compared to ET. During diagnosis, thrombotic and bleeding events were present with no significant difference in frequency among PV, ET, and PMF. Overall survival median was the longest in ET, compared to MF with the lowest survival.
