[Psychopathological features of victimity in hemophiliacs]. / Psikhopatologicheskie osobennosti viktimnosti u bol'nykh, stradaiushchikh gemofiliei.

AIM: To study psychopathological features of victimity in hemophiliacs. MATERIAL AND METHODS: Thirty-one men with hemophilia, aged 17-63 years, mean age 33.43±9.09, illness duration 16-62 years, were included in the study. All patients were admitted to the hospital due to the main disease and its complications. One of the inclusion criteria was a mental disorder with the symptoms of victimity. A clinical/psychopathological method was used in the study. RESULTS AND CONCLUSION: Mental state of the patients depended on the type of personality disorder. Four types of victimity (paranoiac, dissociative, dissocial, dependent) were singled out. Psychopathological features of each type and correlation with the psychiatric diagnosis were described.

Effect of Erythrocyte Density on Blood Clot Properties in Healthy Individuals and in Patients with Hemophilia A.

We measured specific volume and hematocrit of blood clots prepared from the whole blood of patients with hemophilia A and healthy male volunteers. It was shown that in the hematocrit range of 43.5-52.5%, specific volume of the blood clot in hemophilia patients with low level of factor VIII (1-4%) was higher than in volunteers. After injection of factor VIII, specific volume of blood clots in hemophilia patients decreased. Hematocrit of the blood clots derived from the whole blood linearly depended on the mean erythrocyte density in both volunteers (r=-0.74, p=0.01) and patients with factor VIII level of 1-4% (r=-0.95, p<0.0001). The increase in the mean erythrocyte density led to a decrease in blood clot hematocrit. The curve describing blood clot hematocrit as a function of the mean erythrocyte density in hemophilia patients was lower than in healthy volunteers. The increase in factor VIII level was associated with an increase in blood clot hematocrit. The results showed that blood clot hematocrit depends on the mean erythrocyte density, and therefore, hematocrit of the blood clot can be changed by modulating the properties of erythrocyte population.

[Thrombotic events in patients with hemophilia]. / Tromboticheskie oslozhneniia u bol'nykh gemofiliei.

The paper describes 4 clinical cases of thrombotic events (pulmonary embolism, deep vein thrombophlebitis, acute myocardial infarction, ischemic stroke) that have occurred in patients with hemophilia. It discusses the possible causes of their development and methods for their prevention and treatment. Controlled natural hypocoagulation, in which the dose of an administered deficient factor decreases to such an extent that in order to maintain the safe level of hypocoagulation (plasma factor activity is 15-20%; activated partial thromboplastin time is 1.5-2 times normal values), is proposed as one of the treatment options.

Prediction of Intraoperative Blood Loss during Total Knee Arthroplasty in HCV+ and HCV- Patients with Hemophilia A.

We examined HCV+ and HCV- hemophilia A patients with knee arthropathy and hematocrit above 38.5%. The mean density of erythrocytes was studied by the phthalate method, intraoperative blood loss was assessed gravimetrically. The volume of blood loss in HCV+ patients with manifest adhesive process and chronic synovitis varied from 300 to 1900 ml, in patients with moderate adhesive process from 400 to 1500 ml. The volume of blood loss in HCV- patients was 300-800 ml. A positive correlation between the blood loss volume and mean density of erythrocytes was detected. Blood loss >1000 ml during total knee arthroplasty can be expected in patients with hemophilia A with HCV and high mean density of erythrocytes. Blood loss >1000 ml is unlikely in HCV- and HCV+ patients with the mean density of erythrocytes not surpassing the normal values.

[The specific features of pain syndrome in patients with hemophilia]. / Osobennosti bolevogo sindroma u patsientov s gemofiliei.

AIM: to study the nature of pain in hemophilic arthropathy and its prevention ways used by patients with hemophilia. SUBJECTS AND METHODS: A prospective, multicenter, cross-sectional study was conducted to interview 136 patients with hemophilia A or B. The survey included 32 points, including questions about drug and nondrug pain treatment, as well as questionnaires to assess the severity of pain and its impact on daily life (Brief Pain Inventory) and those to identify a neuropathic pain component (PainDetect questionnaire). RESULTS: 83 (75%) patients with hemophilia experienced acute pain associated with bleeding into the large joints; 44 (39%) patients had chronic pain that had lasted longer than six months; 33% assessed the moderate pain experienced in the past 24 hours as severe (more than 5-8 of the 10 scores). In addition to the above, only 32% of the respondents indicated that they had painless intervals within the last 24 hours. 75% of the hemophilia patients mentioned to have limited daily activities. 74% reported their partial or complete disability when pain occurred. 77% of the respondents pointed out that when having pain, they had experienced great difficulty walking. 55% of the hemophilia patients had sleep disorders because of pain. When acute pain occurred, only 91 (81%) respondents injected a factor VIII or IX preparation, 37% of the respondents used narcotic analgesics; 51% received different nonsteroidal anti-inflammatory drugs; 13% took paracetamol. Some patients indicated that they used alcohol and illegal narcotic drugs to relieve pain. Attention is drawn to the fact that the hemophilia patients very frequently took painkillers: 60% of the respondents used analgesics every month; of them 49% had them every week, 11% every day. Most patients (n=83 (74%)) stated that they treated pain (prescribed drugs) themselves; 49 (44%) patients held they were dissatisfied with their pain treatment. When the question as to how the analgesic you used could "remove' pain was asked, only 12 (10%) patients answered that this could fully relieve the pain; 31 (26%) patients told that the pain was not reduced even by half. The study has shown that a neuropathic pain component is very common in hemophilia patients (31%). CONCLUSION: Pain in patients with hemophilia is a serious problem that negatively affects their quality of life, including their working capacity. The prevention and treatment of pain in hemophilia patients should involve the following: organization of educational activities and pain services; adequate treatment of acute pain; detection and prevention of a neuropathic pain component.

Effect of Hematocrit and Erythrocyte Density on Intraoperative Blood Loss in Hemophilia A Patients During Total Knee Arthroplasty.

Intraoperative blood loss during total knee arthroplasty in patients with hemophilia varies over a wide range (from 300 to 3000 ml). The reasons have not been clarified yet. We studied the dependence of intraoperative blood loss during total knee arthroplasty in patients with hemophilia A on hematocrit and mean erythrocyte density. Intraoperational blood loss ≥1000 ml was observed in patients with hematocrit <38.5%. In patients with hematocrit >38.5% this parameter depended on the mean erythrocyte density: in patients with increased erythrocyte density, the risk of intraoperational blood loss ≥1000 ml was higher. The increase in erythrocyte density can serve as an indicator of pathological processes, including the processes modulating hemostasis. It can also be assumed that erythrocytes with higher density change blood flow, which affects platelet adhesion to the damaged endothelium. Hematocrit below the threshold level and mean density of erythrocytes above the normal level can be regarded as risk factor for increased intraoperational blood loss.

[THE ROLE OF THE COMBINATION OF INFLAMMATORY PROCESSES IN THE JOINTS AND ALTERED REGIONAL MICROCIRCULATION IN THE DEVELOPMENT OF HEMOPHILIC ARTHROPATHY].

AIM: To study the role of the combination of inflammatory processes in the joints and altered regional microcirculation as well as factors influencing them in the development of hemophilic arthropathy in 82 patients with recurrent hemarthrosis of the knee and ankle joints by computed IR thermography, laser Doppler flowmetry, and bioimpedancometry for determining fatty and muscle tissue mass. It was shown that deviation of body mass from the normal value were pathogenetically associated with different variants of hemophilic arthropathy. Its risk factors in the patients with continuously recurrent hemarthrosis include epiarticular perfusion, elevated body temperature, low BMI, and muscle mass deficit in excess of 2 kg. 12% of the patients with the history of hemarthrosis-free joints experienced a rise in epiarticular temperature and 6% suffered enhanced perfusion due to latent post-hemorrhagic inflammation.