Introduction. Frequent bleeding with hemophilia significantly worsens the quality of life of patients. The pathogenesis of hemorrhage in hemophilia has not been studied enough, especially at the vascular level, so it is necessary to study microcirculation in this disease. The purpose of the study is to assess the mechanisms of regulation of blood tissue perfusion and the adaptive reserves of the microcirculation system in patients with hemophilia. Materials and methods. Total microcirculation was assessed by laser Doppler flowmetry in 44 patients with hemophilia A between the ages of 14 and 20 years. Severe form of the disease was in 59 % of patients, the average – in 32 %, light – in 9 % of patients. The control group included 26 healthy men aged 14 to 19 years. The sensors recorded blood flow in the index fingers on both sides. In 20 patients with hemophilia А, an occlusion test was performed. The results of the study. In patients with hemophilia, asymmetric changes in microcirculation parameters were detected when measured in the area of the index fingers. At rest in patients with hemophilia, the prevalence of vasospasm was revealed: a decrease in the perfusion index M, an increased blood bypass due to the predominance of myogenic tone. However, neurogenic tone indicators tended to decrease. During occlusive ischemia, vasospasm is slowed down in the first seconds after the onset of exposure to the stress factor. Conclusion. The study revealed a dysregulation of the vascular tone of the microvasculature in young hemophilia patients at rest and under the influence of a stress factor in the form of short-term ischemia. Therefore, with hemophilia from a young age, control of microcirculation is necessary for the timely prevention of both bleeding and cardiovascular pathology associated with vasospasm.
The article is dedicated to contemporary views on the change of endothelial function in the patients with lymphoproliferative disorders prior to, and in the process of, chemotherapeutic treatment. Considering that possibilities of standard examination do not always help identifying subclinical endothelial dysfunction, it is necessary to use specific methods, in particular, to determine the levels of endothelin-1 and vascular endothelial growth factor to monitor endothelial function. The objective of this review is to identify problems and prospects for recognizing early subclinical changes of endothelial function in the patients with lymphoproliferative disorders before and after chemotherapy. Assessing presence and severity of endothelial dysfunction may be useful for determining subclinical stages of cardiovascular damage, stratifying the risk of the patients with confirmed cardiovascular disease, and reducing the likelihood of cardio- and endotheliotoxic effects in patients long after chemotherapy. That is why early detection and immediate therapy of cardiovascular toxicity is currently the most important task in the patients with lymphoproliferative disorders, receiving chemotherapy.
Atypical hemolytic uremic syndrome (aHUS) is a systemic disease, a type of thrombotic microangiopathy (TMA). It is based on uncontrolled activation of the alternative complement pathway of a hereditary or acquired nature, leading to generalized thrombosis in the microvasculature. Chronic activation of the alternative complement pathway leads to the damage of endothelial cells, erythrocytes and platelets and, as a result, to thrombotic microangiopathy and systemic multiorgan damage. Currently, in roughly half of the cases, it is impossible to identify aHUS triggers. Fresh frozen plasma (FFP) is used as first-line drug to reverse the symptoms. It helps to eliminate the deficiency of self-proteins – complement factor H and complement factor I (CFH and CFI), membrane cofactor protein (MCP), and stable and labile proteins – factors of hemostasis, and to stop thrombosis in the microvasculature. FFP administration is a preparatory step before anticomplementary therapy. Disease prognosis is always serious and is associated with severe complications and high mortality. At least 6 % of patients develop multiple organ failure with generalized TMA, injury of the central nervous system, gastrointestinal tract, lungs, and kidneys. The paper describes a clinical case of a patient with aHUS.
Статья посвящена современному представлению о кардиотоксичности, индуцированной химиотерапией у пациентов с гематологической патологией, и методам ее диагностики. Изучение кардиотоксичности является одним из актуальных направлений в онкологии и онкогематологии в настоящее время, так как побочные эффекты химиотерапевтических препаратов на сердечно-сосудистую систему оказывают влияние на качество и продолжительность жизни пациентов, независимо от прогноза, связанного с онкогематологическим заболеванием. В то время как современные стратегии лечения онкогематологических заболеваний позволяют добиваться стойких ремиссий и увеличения продолжительности и качества жизни пациентов, такие успехи могут быть нивелированы повышением смертности в данной группе пациентов за счет развития побочных эффектов химиотерапии применительно к сердечно-сосудистой системе. В настоящем обзоре на современном уровне развития медицинской науки представлены проблемы и перспективы диагностики кардиотоксичности у пациентов с гематологическими заболеваниями на фоне проведения химиотерапевтического лечения. Использование для диагностики кардиотоксичности таких методов, как трехмерная эхокардиография, эхокардиография с определением глобальной продольной деформации миокарда, стресс-эхокардиография с определением контрактильного резерва, определение содержания тропонинов и натрийуретического пептида позволяет обнаруживать проявления кардиотоксичности на более ранних сроках и таким образом снижать смертность у пациентов онкогематологического профиля, получающих химиотерапевтическое лечение от негематологических причин. Ключевые слова: кардиотоксичность, химиотерапевтическое лечение, трехмерная эхокардиография, фракция выброса, глобальная продольная деформация миокарда, стресс-эхокардиография, тропонины Т и I, натрийуретический пептид.
In the present review the cardiovascular complications in patients with chronic myeloid leukemia (CML) receiving tyrosine kinase inhibitors (TKI) are discussed. It covers current views on pathogenesis of TKI cardiovascular toxicity. The pathophysiology of cardiovascular diseases (CVD) is considered as a part of the so-called pathophysiological continuum, i.e. a complex of processes developing at the molecular and cellular levels before clinical symptoms of the above diseases occur. Cardiovascular toxicity of certain TKIs can contribute to progression of pathophysiological processes in CML patients. The study of mechanisms underlying cardiovascular complications of TKI-based therapy is essential for evaluating the risks of their development in each patient. Identification of CVD predictors during TKI-based therapy can allow to elaborate a scheme for cardiovascular monitoring and safe patient management under consideration of individual risks and to avoid severe life-threatening complications.
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