[Methodological recommendations for surgical care in patients with hemophilia A receiving prophylactic therapy with emicizumab]. / Metodicheskie rekomendatsii po okazaniyu khirurgicheskoi pomoshchi u patsientov s gemofiliei A, poluchayushchikh profilakticheskuyu terapiyu emitsizumabom.

Methodological recommendations for surgical care in patients with hemophilia A receiving prophylactic therapy with emicizumab. Recommendations of the expert group. Moscow, 2024.

[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.

[Acute arterial thrombosis in hemophilia with inhibitory antibodies]. / Ostryi arterial'nyi tromboz pri ingibitornoi forme gemofilii.

The authors demonstrate an importance of personalized approach to perioperative hemostatic therapy in a 48-year-old patient with hemophilia A and inhibitory antibodies. Laparoscopic hernia repair and extraction of 15 decayed teeth were performed. Hemostatic therapy included AICC and rFVIIa. Postoperative period was complicated by acute thrombosis of splenic artery and partial spleen infarction. An essential factor in splenic artery thrombosis was increase in blood coagulation potential under rFVIIa administration and depletion of fibrinolytic system (prolongation of XIIa-dependent fibrinolysis from 25 to 75 min) and antithrombin III decrease up to 81%. Cancellation of hemostatic therapy under TEG control ensured fast regression of arterial thrombosis and preservation of spleen. Individual characteristics of patients (compensatory mechanisms of coagulation, comorbidities, clinical changes) should be considered when prescribing hemostatic therapy in hemophilia patients. Perioperative control of all possible coagulation tests (routine and integral) is required for individual selection of hemostatic therapy and decrease of the risk of hemorrhagic and thrombotic complications.

[Inhibitory and mild forms of hemophilia: diagnostic difficulties, surgical complications]. / Trudnosti diagnostiki i khirurgicheskie oslozhneniia pri razlichnykh formakh gemofilii.

AIM: To identify patients with hemophilia who have a high risk of postoperative hemorrhagic complications. MATERIAL AND METHODS: Prospective trial included 69 patients aged 18-71 years (median 29) with congenital hemophilia A and B. They underwent elective and emergency treatment for abdominal and thoracic pathology at the National Medical Research Center for Hematology in 2011-2016. Patients with mild and inhibitory forms of hemophilia were compared with those with severe and moderate forms of hemophilia. There were 50 (73%) patients with severe and moderate forms of hemophilia, 8 (11%) with inhibitory and 11 (16%) patients with mild form. Emergency operations were performed in 18 cases, elective - in 51. RESULTS: Inhibitory form of hemophilia is associated with 1.5 times higher (95% CI, 1.1-3.0) risk of hemorrhagic postoperative complications and death and 3,5 times higher (95% CI 1.7-5.9) risk of redo surgery compared with severe and moderate forms (p<0.05). Risk of hemorrhagic postoperative complications is also higher in patients with mild form of hemophilia compared with severe and moderate forms (1/6 vs. 1/50; p=0.05). CONCLUSION: The risk of postoperative hemorrhagic complications is significantly higher in inhibitory and mild hemophilia compared with severe and moderate forms and associated with hemostatic therapy defects and inadequate assessment of hemostatic disorders. Long-standing haemorrhagic syndrome should be followed by blood clotting system analysis including evaluation of procoagulant activity, presence of inhibitor, and thromboelastography. Decreased levels of albumin (by 2.9-8.6% in our trial) and cholesterol (by 6.5-54.8%) reflects impaired liver function and is sign of unfavorable prognosis. This finding should be considered for surgery and therapy of hemorrhagic manifestations.

[The particularities of acute surgical diseases treatment of abdominal cavity organs in patients with haemophilia].

The experience of treatment of 366 patients with haemophilia who were urgently hospitalized in hеmatological Scientific Center over the last 10 years is presented in the article. There were 114 (31.1%) patients with acute diseases of abdominal cavity organs, 150 (41%) patients with bleeding from upper gastrointestinal tract, 102 (27.9%) patients with acute hematomas of retroperitoneal space. Urgent operations were performed in 48 (22.2%) patients who were hospitalized with clinical symptoms of acute abdomen syndrome. It was developed the criteria of diagnosis and choice of treatment tactic on the basis of the received results. Application of presented algorithms led to improve the quality of urgent surgical care to patients with haemophilia.

[Investigation of ß-endorphin concentrations in patients with hemophilia].

UNLABELLED: AIM. To study changes in the plasma concentration of beta-endorphin (beta-E) in patients with hemophilia A and B (in the presence of bleeding and in the absence of hemorrhagic syndrome) and in whole blood and plasma donors before and after donation and to investigate the factors associated with (beta-E) concentration changes. SUBJECTS AND METHODS: The prospective study of beta-E concentration changes (and related factors) enrolled 38 persons: 12 patients with hemophilia after acute blood loss, 11 patients with hemophilia without hemorrhagic syndrome, and 15 whole blood and plasma donors. beta-E concentrations were measured by enzyme immunoassay. RESULTS: In blood loss, the patients with hemophilia were found to have elevated serum beta-E concentration: 9.6 pg/ml (95% confidence interval (CI), 6.1 to 13.0 pg/ml) versus 5.2 pg/ml (95% CI, 1.4 to 8.9 pg/ml). After donation, the concentration of 3-E in the group of donors was higher than before donation: 7.3 pg/ml (95% CI, 4.9 to 9.7 pg/ml) versus 4.7 pg/ml (95% CI, 3.2 to 6.3 pg/ml). In the group of patients with hemophilia, the elevation of 3-E concentrations is steady-state (lasted at least 10 days); at this time, the beta-E value variability (estimated by mean square deviation) increased as compared with that in remission: 7.7 pg/ml (95% CI, 5.5 to 13.1 pg/ml) versus 2.4 pg/ml (95% CI, 1.7 to 4.4 pg/ml). The above differences are statistically significant (p = 0.05). CONCLUSION: In blood loss, there is an increase in plasma beta-E concentrations in the patients with hemophilia and donors. The increase in beta-E concentrations and the variability of its values were greater in the patients with hemophilia and blood loss than in the donors. The beta-E concentration elevation accompanying hemorrhage is characterized by steadiness in the patients with hemophilia.

[Neurosurgical treatment for chronic subdural hematoma in a patient with chronic autoimmune thrombocytopenic purpura].

Intracranial hemorrhage in patients with chronic autoimmune thrombocytopenic purpura (CATP) is a rare and severe complication of the disease. By taking into account a concomitance of chronic subdural hematoma (CSH) and CATP and no generally accepted approaches to managing the patients with this concomitance, the authors describe a clinical case of mini-invasive CSH drainage in a patient with CATP.

[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.