Применение тромбоэластографии, теста генерации тромбина и кло ттинговых тестов д ля оценки эффективности гемостатической терапии рекомбинантным активированным ф актором VII у больных с ингибиторной формой гемофилии

Introduction . The Problem of the use of recombinant activated factor VII (rFVIIa) in hemophilia patients with inhibitors is the complexity of the laboratory evaluation of the therapy. After rFVIIa  administration standard clothing tests are changing, however, are not normalized. The aim of this study was to investigate the possibility of using standard clothing tests (activated partial thromboplastin time (APTT) and prothrombin time (PT)) to assess the efficiency of hemostatic  therapy with rFVIIa in hemophilia patients with inhibitors. The aim of this work is to investigate the possibility of using standard clothing  tests (activated partial thromboplastin time (APTT), prothrombin time (PT)) to assess the efficiency of hemostatic  therapy with rFVIIa in patients with inhibitory hemophilia. Materials and methods. 20 male hemophilia patients with inhibitors were included in the study. At the time of study inclusion  none patients had signs of bleeding. Plasma levels of FVIII were < 1  %, FVIII inhibitor titers were from 5 BU/ml to 463 BU/ml. All  patients received rFVIIa (Coagil-VII) at a dose of 90 μg/kg. Before  the administration of rFVIIa and then in 15, 30 and 60 min, 2 and 24 h the APTT, the PT, the endogenous thrombin potential (ETP) and thromboelastography (TEG) parameters (reaction time – R, maximum amplitude – MA) were evaluated. Results. Before rFVIIa administration all patients had prolonged APTT. 15 min after administration of rFVIIa APTT shortened and remained shorter than baseline level, but 2 times longer than  normal ranges during 2 hours. The PT also significantly decreased in  15 min after the rFVIIa administration. Prior to treatment patients had minimal to no clotting detectable by TEG. Administration of rFVIIa led to normalization of TEG traces in the most of the patients  in 15 min. Elevated by TEG hemostatic effects persisted for 2 h. The  ETP increased in 15 min after rFVIIa administration. This increase of  ETP persisted for 60 min. There were strong correlations between R  and APTT (r = 0.74; p = 0.001), between MA and APTT (r = 0.70),  between PT and R (r = 0.79; p = 0.01), PT and MA (r = 0.76; p =  0.01). There were no correlations between ETP and APTT, and  between ETP and R. The shortening of the APTT after rFVIIa administration for 17 s and more or for 22 % and more from the baseline levels were associated with the normalization of R and MA.  Changes of the PT poorly allowed to discriminate normal values of  TEG. Conclusion . Shortening of the APTT after rFVIIa administration in hemophilia patients with inhibitor for 17 s and more or for 22 % and more from the initial value can be used to assess the hemostatic efficiency of rFVIIa therapy.