Background. Internationally published data about the course of COVID-19 in patients with congenital bleeding disorders (CBDs) are limited. There are questions about how COVID-19 affects the course of CBDs and, conversely, how CBDs affect the course of coronavirus infection.Aim — to analyze the course of COVID-19 in patients with CBDs in Russia.Materials and methods. A cross-sectional survey was conducted at the National Medical Research Center for Hematology (Moscow) for the period from June 25 to July 31, 2022. A cluster of 187 patients from different regions and cities of Russia were interviewed with a questionnaire based on survey administration software (Google forms) containing 27 questions.Results. COVID-19 affected 115 (62 %) of 187 surveyed patients and 22 (19 %) patients suffered from coronavirus infection twice. Hospitalization was required for 14 (12 %) patients with an average age of 42 years (10 patients with severe hemophilia A, 1 patient with moderate hemophilia B, 2 patients with von Willebrand disease and 1 patient with hypoproconvertinemia). During COVID-19 bleeding was observed in 9 (8 %) patients and was represented by hemarthrosis, ecchymosis, hematomas, nosebleeds, menorrhagia, hemorrhoidal bleeding, hemorrhage in the eyeball. There were no cases of thrombosis among 115 COVID-19 patients. Among patients with CBDs who had COVID-19, compared with patients who had not COVID-19, von Willebrand disease was statistically significantly more common (p = 0.04). Changes in the course of CBD after COVID-19 were noted by 21 (18 %) of 115 patients: 11 (10 %) of those who were ill noted increased joint pain, 9 (8 %) complained of joint pain that had not been previously experienced; 10 % of patients described changes of hemorrhagic syndrome.Conclusion. The probability of hospitalization of patients with CBDs and COVID-19 older than 40 is statistically significantly higher. Von Willebrand disease can be considered as a potential risk factor for COVID-19. Given the absence of cases of thrombosis in the interviewed group of patients, the results of the study suggest that the presence of hypocoagulation in patients with CBDs may be a protective pathophysiological mechanism that prevents the development of COVID-19-associated thrombotic complications.
The paper describes a case of autoimmune hemolytic anemia (AIHA) in a 27-year-old woman whose examination revealed mesenteric teratoma. AIHA was characterized by a hypertensive crisis and a temporary response to corticosteroid therapy that was complicated by the development of somatogenic psychosis and discontinued. A relapse of hemolysis developed 6 months later. The patient underwent laparoscopic splenectomy and removal of mesenteric root teratoma. Immediately after surgery, a hematological response was obtained as relief of hemolysis and restoration of a normal hemoglobin level. There is a sustained remission of AIHA for the next 16 months.
What keeps regular blood donors coming back, despite the inconvenience and discomfort, thereby maintaining the community's blood supply? We approached 494 people waiting to give blood, 229 Americans and 265 Russians, with a survey that assessed their motivations to donate, their contextual emotions, and their future donation intentions. The Russian sample was older, and many were regulars at the donation centre. The U.S. sample was younger, attending a 3‐day college blood drive. Multigroup path analysis results showed that, as hypothesised, in both samples, autonomous donation motivations predicted contextual emotions and future donation intentions. Furthermore, contextual emotions partially mediated the motivation‐to‐future intentions effect. Small differences in results for the two samples, as well as differences in the two national systems for maintaining blood supplies, are discussed.
Background. Combined injuries involving brain damage represent the most severe and life-threatening conditions in hemophilia patients. These injuries are characterised by specific situational and behavioural circumstances indicating the presence of victim behaviour in such patients.Aim. To analyse the influence of victim behaviour in hemophilia patients on the formation of combined neurosurgical trauma and the choice of neurosurgical and traumatological treatment approaches.Materials and methods. Twenty five patients (20 patients with hemophilia A and 5 patients with hemophilia B) were included in the study. The patients suffered the following injuries: craniocerebral injuries — 68 (100.0 %); bone fractures — 18 (26.6 %); hematomas of the soft tissues of the face, upper and lower extremities, as well as bruised, lacerated wounds — 50 (73.4 %).Results. The following types of victim behaviour were identified in the hemophilia patients: paranoid — 7 (28.0 %), dependent — 8 (32.0 %), dissociative — 6 (24.0 %) and antisocial — 4 (16.0 %). The patients underwent hemostatic therapy with coagulation factor VIII or IX concentrates and surgical (neurosurgical and/or traumatological) treatment of the injuries associated with victim behaviour. In 51 (75.0 %) cases there was delayed medical care, which was the reason for the complicated course of the post-traumatic period. It was revealed that the best treatment results in patients with severe injuries, including craniocerebral traumas, were achieved in cases where medical assistance was provided in the first three hours after injury. As a result of the treatment, the majority of the patients demonstrated regression of the clinical manifestations of the injury.Conclusions. The proposed tactics for the diagnosis and neurosurgical/traumatological treatment of hemophilia patients with signs of victim behaviour, in whom combined brain injuries are detected, includes a comprehensive assessment of medical history data, clinical and laboratory examination, as well as determination of diagnostic criteria for the choice of patient-specific surgical techniques. It is proposed to use the number of patients’ return visits related to their victim behaviour as a quantitative assessment of the degree of victimization.Conflict of interest: the authors declare no conflict of interest.
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