Patients with myeloproliferative diseases (MPD) are noted to be at high risk for portal thromboses. This problem gives rise to disability if it is untimely treated or resistant to therapy. The paper gives the experience of the Outpatient Department of the Hematology Research Center, Ministry of Health of the Russian Federation, in using antithrombin III in MPD patients (3 patients with primary myelofibrosis, 3 with essential thrombocythemia) and acute and subacute portal vein thromboses resistant to therapy with direct anticoagulants. In all 5 cases, the use of antithrombin III in combination with low-molecular-weight heparin showed a positive clinical effect as rapid relief of pain syndrome and comparatively early (3-week to 1.5-2-month) recanalization of thrombosed vessels. Three clinical cases are described in detail.

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.

Recommendations for the diagnosis and therapy of Rh-negative myeloproliferative diseases (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 Hematologist and Transfusiologist of the Ministry of Health of Russia, Head, Hematology Research Center). The aim of these recommendations is standardization of the diagnostic and therapeutic approaches in Russia. The methodological approaches are based on the conclusive medicine philosophy and the recommendations of the European Leukemia NET (ELN), National Cancer Control Net (NCCN; USA), International Working Group "Myeloproliferative Neoplasm Research and Treatment" (IWG-MRT), and on the experience gained in Russia on the treatment and follow-up of patients with myeloproliferative diseases. The recommendations are developed by a Multicenter research group for studies of myeloproliferative diseases. Scientists from 10 leading institutions contributed to development of the clinical recommendations.

The paper describes a case of practically simultaneous development of the hemolytic-uremic syndrome (HUS) and the catastrophic antiphospholipid syndrome (CAPS) complicated by mesenteric vessel thrombosis and small bowel necrosis. Multimodality treatment comprising volume plasmapheresis, fresh frozen plasma transfusion, hemodialysis, anticoagulant and disaggregant therapy could relieve thrombogenic events, such as pulmonary artery thromboembolism and intestinal necrosis.

AIM:To define an optimal diagnostic and therapeutic algorithm when the acute abdominal syndrome occurs in hematological patients.MATERIALS AND METHODS:The results of 145 emergency surgeries made in 2006-2008 for acute abdominal syndrome were studied in patients with blood system diseases.RESULTS:Clinical manifestations of acute abdominal syndrome emerge in 1-1.4% of all the patients treated at the Hematology Research Center, Russian Academy of Medical Sciences. There is a need for surgery in 0.5-0.7% of all the patients admitted. In this group of patients, annual postoperative mortality is 12-16%.CONCLUSION:The routine algorithm for a diagnostic search in hematological patients with acute abdominal syndrome can lead to both hyperdiagnosis and unwarranted surgery, and incorrect choice of expectant policy as well.

Relationship between hyperhomocysteinemia and reproductive loss and correction of this condition during pregnancy. S. S. Mondoeva(2), G. A. Sukhanova(1), N. M. Podzolkova(2), A. A. Levina(1), S. A. Vasilyev(1). Hematology Research Center(1); Russian Medical Upgrading Academy(2), Moscow. Testing of 150 women with a history of obstetrical complications (miscarriages, antenatal fetal death, small-for-date fetuses, failure of extracorporeal fertilization) for hereditary and acquired thrombophilia detected hyperhomocysteinemia (HHC) in 44% (n = 66). In 92.4% (n = 61) of these the increase in homocystein level was caused by MTHFT gene C677-T and MTRR gene A66G polymorphisms. A specific feature of the majority of HHC patients was reproductive loss (fetal death and endothelial dysfunctions). Angiovit treatment during pregnancy led to normalization of homocystein level.

Clinical manifestations of subleukemic myelosis, which debuted with Budd-Chiari syndrome in a young female patient, are presented. The cytoreductive therapy should be spent simultaneously with anticoagulant therapy.

Published data on the blood clotting final stage physiology and pathophysiology are reviewed. The genetic structure of fibrinogen molecule is described, with characteristics of its functional properties, stages and mechanisms of fibrinogenesis processes. The role and significance of fibrinogen in the maintenance of the hemostatic function are characterized.

AIM:To assess incidence of hyperhomocysteinemia (HHC) in patients with chronic myeloproliferative diseases (CMPD) and to analyse possible correlation between an elevated concentration of plasma homocystein (HC) and thrombotic complications.MATERIAL AND METHODS:The trial enrolled 61 patients: 39 CMPD patients with thrombotic complications and free of them, 22 nonhematological patients with thrombosis. The control group consisted of 40 healthy donors. The examination protocol included determination with standard methods of HC plasma concentration, platelet and plasma components of hemostasis, mutation of factor V Leiden gene, prothrombin and methylenetetrahydrofolate reductase (MTHFR).RESULTS:Mean HC concentration in the serum in CMPD patients was 19 +/- 1.7 mcmol/l which appeared higher than in healthy donors (12 +/- 1.3 mcmol/l). The highest HC was in patients with subleukemic myelosis (SLM)--23 +/- 2.3 mcmol). No difference in HC concentration in plasma was observed in CMPD carriers of homo- or heteroxygous mutation of C667T gene or CMPD patients without the mutation. In CMPD content of factor VIII was higher in HHC than in normal HC (222 +/- 26.5 and 116 +/- 20%, respectively, p = 0.002). For von Willebrand factor 202 +/- 15.6 and 120 +/- 14.6%, respectively (p < 0.003). HC reduction in response to vitamin therapy was the greater the higher its initial level was.CONCLUSION:There is correlation between HHC and thrombosis in CMPD patients. HC concentration may depend on the proliferative stage of CMPD. As HC is a significant independent factor of thrombotic complications risk, it is necessary to detect and treat HHC.

Effective transplantation of allogenic bone marrow was carried out in a female patient with B-cellular acute lymphoblastic leukemia, hereditary thrombophilia, and relapsing thromboses during controlled antithrombotic preventive treatment.