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Information × Registration Number 0217U000481, 0115U000127 , R & D reports Title To investigate the type inhibitors of coagulation and their pathogenesis in patients with congenital and acquired hemophilia, and antiphospholipid syndrome popup.stage_title Head Loginsky Volodymyr Eustakhovych, Доктор медичних наук Registration Date 06-02-2017 Organization LVIV RESEARCH INSTITUTE OF BLOOD PATHOLOGY AND TRANSFUSIONAL MEDICINE popup.description2 The object of study - 61 patients with hemorrhagic syndrome aged from 6 months to 63 years have been diagnosed and treated in clinic of SI "Institute of Blood Transfusion Medicine and Medical Sciences of Ukraine" (Lviv). Aim: To set the type of inhibitor and its pathogenetic and prognostic value in the clinical course of the disease and the effectiveness of treatment of patients with hemophilia, immune coagulopathy and patients with antiphospholipid syndrome (APS) by investigation the kinetic of inhibitory antibodies in response to adding normal plasma. Clinical and laboratory studies included clinical examination and tests of coagulation, hematology, biochemical. We used Micros 60 hematology analyzer T number 81473, biochemical analyzer Point + 180, SR 1907.1674, semi-automatic coagulometer Helena-C4 S4-2489 number. As a result of research among 61 patients with hemorrhagic syndrome and isolated prolonged activated partial thromboplastin time (APTT) we received the next distribution of patients: hemophilia A - 34.4%, hemophilia B - 11,5%, hemophilia A with inhibitor - 13,1%, acquired hemophilia A - 6,5%, acquired hemophilia B 1,6% , von Willebrand disease 4,9%, antiphospholipid syndrome (APS) - 27,9%. According to results we provided an laboratory algorithm, which includes two types of corrective tests and specifying tests which can differentiate deficiency of factors VIII(IX) and type I and type II inhibitors. We found II type inhibitor in 3 acquired hemophilia patients, based on this algorithm, which was a typical autoimmune antibodies. Immune coagulopathy is a rare autoimmune disease occurs independently of substitution therapy and causes hemorrhagic complications, including severe life-threatening bleeding. As a control-bleeding agent , the drug of choice is recombinant activated factor VII, FVIII (IX) concentrates. As the treatment to eradicate the inhibitor immunosuppression (prednisone, cytostatics) should be commenced in all patients as soon as the diagnosis has been made. Another effective method is plasmapheresis. The optimal strategy for inhibitor eradication is unknown. The main method of prevention of immune inhibitor is treating the underlying disease. Product Description popup.authors Дзісь Р.П. Красівська В.В. Стасишин О.В. Тушницький О.М. popup.nrat_date 2020-04-02 Close