NATIONAL СLINICAL RECOMMENDATIONS FOR DIAGNOSIS AND THERAPY OF PH-NEGATIVE MYELOPROLIFERATIVE NEOPLASMS (POLYCYTHEMIA VERA, ESSENTIAL THROMBOCYTHEMIA, PRIMARY MYELOFIBROSIS) (EDITION 2018)

Specialists-hematologists constantly need to update their knowledge in connection with the rapid development of hematology and the introduction of new methods of diagnosis and treatment into practice.

Recommendations for the diagnosis and therapy of Rhnegative myeloproliferative neoplasms (polycythemia vera, essential thrombocythemia, primary myelofibrosis, unclassified myeloproliferative disease, postpolycythemia and postthrombocytemic myelofibrosis) are developed by the research group for studies of myeloproliferative diseases at the initiative of the Russian National Hematological Society (Chairman: Prof. V. G. Savchenko, Chief Extraordinary Expert for Hematology of the Ministry of Health of Russia, Academician, Director General of the National Research Center for Hematology).

The aim of these recommendations is standardization of the diagnostic and therapeutic approaches in Russia. The methodological approaches are based on the recommendations of the Russian expert council (leading specialists of 10  hematological centers of the Russian Federation) for diagnosis and treatment of patients with classical Ph-negative myeloproliferative neoplasms, Russian experience in management of patients and diagnostic criteria approved by WHO in 1017 and the recommendations of the European Leukemia NET (ELN), National Cancer Control Net (NCCN; USA), International Working Group for Myeloproliferative Neoplasm Research and Treatment (IWG-MRT).

The draft clinical guidelines were reviewed on November 11, 2013 at a meeting of the Expert Group on Myeloproliferative Diseases. The discussion was attended by leading experts of 10 hematology centers in Russia. The project was approved at the meeting of the Profile Commission on the specialty “Hematology” on March 3, 2014. At the II Congress of Hematology on April 12, 2014, clinical guidelines for the diagnosis and treatment of classical Ph-negative inventories were approved.

Clinical guidelines are a dynamic document. National Clinical Guidelines for the diagnosis and treatment of classic Ph-negative myeloproliferative diseases are updated once every two years. In 2017 clinical recommendations approved at the III Congress of Hematology of Russia on April 15, 2016 were published. This edition is an updated version approved at the IV Congress of Hematology of Russia on April 13, 2018.

The recommendations are intended for hematologists, chemotherapists, health administrators, medical students. 

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