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Aim. To study hemostasis disorders in patients with de novo acute leukemias (AL) prior to chemotherapy. Materials & Methods. The study enrolled 107 patients with newly diagnosed AL, aged 18-80 years and treated at the National Research Center for Hematology. Acute lymphoblastic leukemia (ALL) was identified in 37 patients, acute myeloid leukemia (AML) was diagnosed in 46 patients, and acute promyelocytic leukemia (APL) was reported in 24 patients. Hemorrhagic and thrombotic complications were analyzed; platelet count, APPT, prothrombin and fibrinogen concentration were determined; thromboelastography (TEG; native tests, functional fibrinogen tests) and rotation thromboelastometry (ROTEM; EXTEM, INTEM, FIBTEM, APTEM) were performed. The data were statistically processed using SAS 9.4 software. Results. At AL onset hemorrhagic syndrome was detected in 34 (32 %) out of 107 patients. It was manifested by petechia (n = 16), subcutaneous hematomas (n = 12), gingival (n = 10) and nose (n = 6) bleeding, uterine bleeding (n = 2), hematuria (n = 2), gastrointestinal bleeding (n = 1), brain hemorrhage (n = 6), and periorbital hematoma (n = 1). According to TEG and ROTEM hypocoagulation was more common in APL patients. Hyperfibrinolysis could be detected using only ROTEM in 54 % of APL patients, in 8 % of ALL and 4 % of AML patients. Compared to other AL patients those with APL showed different parameters of fibrinogen concentration of < 1.75 g/L (sensitivity 83.3 %, specificity 83.13 %), D-dimer concentration of > 2686 pg/L (sensitivity 72.73 %, specificity 64.79 %), MCFFIBTEM < 12.5 mm (sensitivity 80 %, specificity 80 %), and MAFF < 9.7 mm (sensitivity 86.96 %, specificity 90.12 %). Conclusion. The parameters that distinguish APL from other categories of AL patients are hypofibrinogenemia, higher D-dimer concentration, ROTEM changes, and hyperfibrinolysis.

Introduction and Objectives: Inherited FVII deficiency is a rare hemorrhagic disorder.Clinical bleeding does not correlate with the level of FVII plasma activity. FVII involves in the extrinsic coagulation pathway. ROTEM is point of care method. EXTEM is one of ROTEM screening tests. EXTEM analyses the extrinsic pathway of coagulation. Aim of the study was to evaluate hemostatic effect and safety of rFVIIa treatment in pts with inherited FVII deficiency. Materials and Methods: The results of rFVIIa treatment were investigated in 4 pts (1 male, 3 females) with inherited FVII deficiency. Pts were treated with rFVIIa before gynecological (2), abdominal (1) and orthopedic surgery (1). Pts received 30 mcg/kg of rFVIIa (Koagil VII, Generium, Russia) before surgery. Plasma FVII activity, fibrinogen, INR, APTT, ROTEM and thromboelastography (TEG) parameters were investigated before rFVIIa administration, then in 15 minutes, in 2 hours, in 6 hours and in 12 hours. Results: Before rFVIIa administration median plasma FVII activity was low, CT EXTEM was long and INR was greater than 3. TEG, APTT and fibrinogen were in normal ranges (table 1, fig. 1). Fifteen minutes after rFVIIa administration FVII:C activity increased, INR and CT EXTEM decreased. Hemostatic effect of rFVIIa remained during 12 hours. During this period CT EXTEM correlated with INR (r = 0.97, p Conclusion: CT EXTEM allowed to evaluate hemostatic effect of rFVIIa in operating room and good correlated with INR in pts with inherited FVII deficiency. Disclosures No relevant conflicts of interest to declare.

Introduction and Objectives: MV in thrombocytopenic pts could be complicated by life threatening tracheobronchial bleeding. The systemic treatment with tranexamic acid or rFVIIa increases the risk of thromboembolic events. Presented in airspace tissue factor (TF) reacts as a cofactor in augmenting the activity of FVIIa 1000-fold. Topical treatment of the inhalation with rFVIIa has not been studied yet. The aim of the study was to evaluate hemostatic effect of rFVIIa inhalation for treatment of the tracheobronchial bleeding in MV thrombocytopenic pts. Materials and Methods: The results of rFVIIa inhalation were investigated in 5 acute leukemia pts (2 males, 3 female). All pts were mechanically ventilated through tracheostomy tube and had life threatening tracheobronchial bleeding. Before intervention all pts had low platelet count 50-120х109/l (median 65 х109/l), lowered Quick prothrombin (55-64%, median 58%), prolonged APTT (55-57s). Results. All pts received rFVIIa as an inhalation over five to ten minutes. For inhalation rFVIIa (Koagil VII, Generium, Russia, Vial of 2.4 mg) was diluted in 10 ml of isotonic saline. If, after 20 min, there is no response to rFVIIa second dose of rFVIIa (2.4 mg) as an inhalation was administrated. Bleeding was stopped in 3/5 pts, in 2 pts the intensity of bleeding decreased. There were no changes in APTT and Quick prothrombin registered. Median plasma FVII activity before inhalation was 82%, after inhalation - 73%. There were no changes on the TEG (figure 1). Thus, despite local hemostatic effect we did not registered systemic effects of rFIIa after inhalation Conclusion. Topical treatment of the tracheobronchial bleeding with rFVIIa inhalation provided hemostatic effect. There were not be systemic adverse effects in as much as the alveolocapillary membrane does not allow the transmembranous passage of rFVIIa. Disclosures No relevant conflicts of interest to declare.

The article describes a rare clinical case of a patient with previously undiagnosed von Willebrand disease and basal meningioma; an intracranial neurosurgical intervention was complicated by delayed intracranial hematomas, both at the resected tumor site and distantly. The diagnosis of von Willebrand disease was established only after special hematology tests and only after surgery. Despite the use of specific therapy, the patient died due to intracranial hemorrhagic complications in the postoperative period. The paper discusses the problem of preoperative diagnosis of asymptomatic hemostasis disorders in neurosurgical patients and potential ways of its solution.Дано описание редкого клинического наблюдения: у пациента с ранее не диагностированной болезнью Виллебранда и базальной менингеомой проведенное интракраниальное нейрохирургическое вмешательство осложнилось формированием отсроченных интракраниальных гематом как на месте удаленной опухоли, так и дистантно. Диагноз болезни Виллебранда был установлен только в результате специального исследования в гематологической лаборатории и только после операции. Несмотря на применение специфической терапии, пациент погиб от интракраниальных геморрагических осложнений в послеоперационном периоде. В работе обсуждаются проблема предоперационной диагностики скрытых нарушений гемостаза у нейрохирургических больных и возможные пути ее решения.