[Surgery in patients with severe or inhibitory hemophilia A under prevention injection of emicizumab]. / Khirurgicheskie vmeshatel'stva u bol'nykh tyazheloi ili ingibitornoi formoi gemofilii A na fone profilakticheskogo vvedeniya emitsizumaba.

OBJECTIVE: To determine the features of preoperative hemostatic therapy and laboratory control in patients with severe and inhibitory forms of hemophilia A under preventive treatment by FVIII. MATERIAL AND METHODS: Four patients with severe and inhibitory forms of hemophilia A underwent surgery between 2021 and 2022. All patients received Emicizumab (the first monoclonal drug for «non-factor¼ therapy of hemophilia) for prevention of specific hemorrhagic signs of hemophilia. RESULTS: Surgical intervention under preventive Emicizumab therapy was essential. Additional hemostatic therapy was not carried out or performed in reduced mode. There were no hemorrhagic, thrombotic or other complications. Thus, the so-called «non-factor¼ therapy is one of the variants for uncontrollable hemostasis in patients with severe and inhibitory forms of hemophilia. CONCLUSION: Preventive injection of Emicizumab ensures certain buffer of hemostasis system and stable lower limit of coagulation potential. This is the result of stable concentration of Emicizumab when used in any of the registered forms regardless of age and other individual characteristics. The risk of acute severe hemorrhage is excluded, while the probability of thrombosis is not increased. Indeed, FVIII has higher affinity than Emicizumab and displaces Emicizumab from coagulation cascade that does not result summation of total coagulation potential.

[Therapy with high-doses of antithrombin III in patients with septic shock and agranulocytosis].

OBJECTIVE: The aim of the study was to assess efficacy of high-doses ofantithrombin 111 (AT) for treatment of septic shock in patients with an agranulocytosis. DESIGN: Prospective, controlled study. PATIENTS: 29 patients from 18 to 74 years old, with blood diseases complicated with septic shock Dates of study: from 2006 to 2012. METHODS: The patients were randomized into two groups. Group-1 included 14 patients, who did not receive AT and group-2 included 15 patients who received AT. RESULTS: Demographic indicators, condition severity according to APACHE II, level of thrombocytopenia, levels ofplasma procalcitonin, interleukin-6 (IL-6) and C-reactive protein (CRP) were the same in both groups. Level of AT was decreased in both groups; however it was higher in the group-1 (50% vs. 60%, p < 0.05). In the group-1, microorganisms were found in the blood of 9 patients. In the group-2, the microorganisms were found in the blood of 11 patients. Inflammation markers were decreased after the treatment of septic shock in both groups (p<0.05). The decreasing of procalcitonin in group-1 was from 43.8 to 1 ng/ml in 14 days and from 12.8 to 1.6 ng/ml in 7 days in group-2. The decreasing of CRP in group-1 was from 224 to 114 mg/l in 7 days and from 146 to 60 mg/l in 14 days in group-2. The decreasing of IL-6 in group-1 was from 1617 to 100 pg/ml in 3 days and from 5895 to 77 pg/ml in 7 days in group-2. A level of AT was increased only in group-2 (under 12% per day). 28-day survival was higher in group-2 (60 +/- 13% vs. 45 +/- 13%, p<0.05). We did not find any complications of the treatment with AT concentrate. CONCLUSION: Treatment of septic shock with high-doses of antithrombin III was effective and safe in patients with an agranulocytosis.

[Acute abdominal syndrome in blood system diseases].

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.

Influence of several chemical reagents on lymphocytic choriomeningitis and Tacaribe viruses.

The sensitivity of lymphocytic choriomeningitis virus (LCMV) and Tacaribe virus to several chemical reagents was investigated. The viruses were sensitive to lipid solvents (ether, chloroform) and to detergents (sodium desoxycholate and Triton X-100); they were rapidly inactivated by formalin, beta-propiolactone, hydrogen peroxide and chloramine B. The results obtained contribute to a more complete characterization of the biological features of the arenavirus group and may be useful for scientific research and manufacturing of viral preparations.