Antónia Hatalova1, Jiri Schwarz2, Mirjana Gotic3, Miroslav Penka4, Mikulas Hrubisko1, Rajko Kusec5, Miklós Egyed6, Martin Griesshammer7,8, Maria Podolak-Dawidziak9, Andrzej Hellmann10, Sergiy Klymenko11, Emilia Niculescu-Mizil12, Petro E Petrides13, Sebastian Grosicki14, Matjaz Sever15, Nathan Cantoni16, Jürgen Thiele17, Dominik Wolf18,19, Heinz Gisslinger20. 1. Clinic of Hematology and Blood Transfusion, University Hospital, Faculty of Medicine, Medical School Comenius University, Slovak Medical University, Bratislava, Slovakia. 2. Clinical Section, Institute of Hematology and Blood Transfusion, Institute of Clinical and Experimental Hematology, Charles University, Prague, Czechia. 3. Clinic for Hematology Clinical Center of Serbia, Medical Faculty University of Belgrade, Belgrade, Serbia. 4. Department of Clinical Hematology, Masaryk University Hospital, Brno, Czechia. 5. Department of Hematology, Dubrava University Hospital, University of Zagreb, Medical School, Zagreb, Croatia. 6. Department of Hematology, Somogy County Mór Kaposi General Hospital, Kaposvár, Hungary. 7. Department of Hematology, Oncology and Palliative Medicine, Johannes Wesling Academic Medical Center, Minden, Germany. 8. University Clinic for Hematology, Oncology and Palliative Medicine, Johannes Wesling Medical Center Minden, University of Bochum, Bochum, Germany. 9. Department of Hematology, Blood Neoplasms and Bone Marrow Transplantation, Wróclaw Medical University, Wróclaw, Poland. 10. Department of Hematology and Transplantology, Medical University Hospital, Gdaňsk, Poland. 11. Department of Medical Genetics, State Institution National Research Center for Radiation Medicine of National Academy of Medical Sciences of Ukraine, Kyiv, Ukraine. 12. Provita Diagnosis and Treatment Center, Bucharest, Romania. 13. Hematology Oncology Center Munich, Ludwig-Maximilian's University, Munich, Germany. 14. Department of Cancer Prevention, Public School of Health, Silesian Medical University, Katowice, Poland. 15. Department of Hematology, University Clinical Center, Ljubljana, Slovenia. 16. Division of Hematology, University Clinic of Medicine, Kantonsspital Aarau, Switzerland. 17. Institute of Pathology, University of Cologne, Cologne, Germany. 18. Department of Internal Medicine V, Hematology & Oncology, Innsbruck Medical University, Innsbruck, Austria. 19. Medical Clinic 3, Oncology, Hematology and Rheumatology, University Hospital of Bonn, Bonn, Germany. 20. Division of Hematology and Blood Coagulation, Department of Internal Medicine I, Medical University of Vienna Hospital, Vienna, Austria.
Abstract
OBJECTIVES: To present the Central European Myeloproliferative Neoplasm Organisation (CEMPO) treatment recommendations for polycythaemia vera (PV). METHODS: During meetings held from 2015 through 2017, CEMPO discussed PV and its treatment and recent data. RESULTS: PV is associated with increased risks of thrombosis/thrombo-haemorrhagic complications, fibrotic progression and leukaemic transformation. Presence of Janus kinase (JAK)-2 gene mutations is a diagnostic marker and standard diagnostic criterion. World Health Organization 2016 diagnostic criteria for PV, focusing on haemoglobin levels and bone marrow morphology, are mandatory. PV therapy aims at managing long-term risks of vascular complications and progression towards transformation to acute myeloid leukaemia and myelodysplastic syndrome. Risk stratification for thrombotic complications guides therapeutic decisions. Low-risk patients are treated first line with low-dose aspirin and phlebotomy. Cytoreduction is considered for low-risk (phlebotomy intolerance, severe/progressive symptoms, cardiovascular risk factors) and high-risk patients. Hydroxyurea is suspected of leukaemogenic potential. IFN-α has demonstrated efficacy in many clinical trials; its pegylated form is best tolerated, enabling less frequent administration than standard interferon. Ropeginterferon alfa-2b has been shown to be more efficacious than hydroxyurea. JAK1/JAK2 inhibitor ruxolitinib is approved for hydroxyurea resistant/intolerant patients. CONCLUSIONS: Greater understanding of PV is serving as a platform for new therapy development and treatment response predictors.
OBJECTIVES: To present the Central European Myeloproliferative Neoplasm Organisation (CEMPO) treatment recommendations for polycythaemia vera (PV). METHODS: During meetings held from 2015 through 2017, CEMPO discussed PV and its treatment and recent data. RESULTS: PV is associated with increased risks of thrombosis/thrombo-haemorrhagic complications, fibrotic progression and leukaemic transformation. Presence of Janus kinase (JAK)-2 gene mutations is a diagnostic marker and standard diagnostic criterion. World Health Organization 2016 diagnostic criteria for PV, focusing on haemoglobin levels and bone marrow morphology, are mandatory. PV therapy aims at managing long-term risks of vascular complications and progression towards transformation to acute myeloid leukaemia and myelodysplastic syndrome. Risk stratification for thrombotic complications guides therapeutic decisions. Low-risk patients are treated first line with low-dose aspirin and phlebotomy. Cytoreduction is considered for low-risk (phlebotomy intolerance, severe/progressive symptoms, cardiovascular risk factors) and high-risk patients. Hydroxyurea is suspected of leukaemogenic potential. IFN-α has demonstrated efficacy in many clinical trials; its pegylated form is best tolerated, enabling less frequent administration than standard interferon. Ropeginterferon alfa-2b has been shown to be more efficacious than hydroxyurea. JAK1/JAK2 inhibitor ruxolitinib is approved for hydroxyurea resistant/intolerant patients. CONCLUSIONS: Greater understanding of PV is serving as a platform for new therapy development and treatment response predictors.
Authors: Philipp Kaiser; Andreas Zuckermann; Johann Horvat; Franz Lederer; Heinz Gisslinger; Franz Gremmel; Paul Simon; Dominik Wiedemann; Martin Andreas Journal: J Card Surg Date: 2020-07-15 Impact factor: 1.620