Unlike classic APS, CAPS causes multiple microthrombosis due to an increased inflammatory response, known as a “thrombotic storm”. CAPS typically develops after infection, trauma, or surgery and begins with the following symptoms: fever, thrombocytopenia, muscle weakness, visual and cognitive disturbances, abdominal pain, renal failure, and disseminated intravascular coagulation. Although the presence of antiphospholipid antibodies in the blood is one of the diagnostic criteria, the level of these antibodies can fluctuate significantly, which complicates the diagnostic process and can lead to erroneous interpretation of rapidly developing symptoms. Triple therapy is often used to treat CAPS, which includes the use of anticoagulants, plasmapheresis, and high doses of glucocorticosteroids and, in some cases, additional intravenous immunoglobulins. The use of LMWH is recommended as the drug of choice due to its anti-inflammatory and anticoagulant properties. CAPS is a multifactorial disease that requires not only an interdisciplinary approach but also highly qualified medical care, adequate and timely diagnosis, and appropriate prevention in the context of relapse or occurrence of the disease. Improved new clinical protocols and education of medical personnel regarding CAPS can significantly improve the therapeutic approach and reduce mortality rates.

Newborns are the most vulnerable patients for thrombosis development among all children, with critically ill and premature infants being in the highest risk group. The upward trend in the rate of neonatal thrombosis could be attributed to progress in the treatment of severe neonatal conditions and the increased survival in premature babies. There are physiological differences in the hemostatic system between neonates and adults. Neonates differ in concentrations and rate of synthesis of most coagulation factors, turnover rates, the ability to regulate thrombin and plasmin, and in greater variability compared to adults. Natural inhibitors of coagulation (protein C, protein S, antithrombin, heparin cofactor II) and vitamin K-dependent coagulation factors (factors II, VII, IX, X) are low, but factor VIII and von Willebrand factor are elevated. Newborns have decreased fibrinolytic activity. In the healthy neonate, the balance is maintained but appears more easily converted into thrombosis. Neonatal hemostasis has less buffer capacity, and almost 95% of thrombosis is provoked. Different triggering risk factors are responsible for thrombosis in neonates, but the most important risk factors for thrombosis are central catheters, fluid fluctuations, liver dysfunction, and septic and inflammatory conditions. Low-molecular-weight heparins are the agents of choice for anticoagulation.

The rate of thrombosis and disseminated intravascular coagulation (DIC) has been increasing in COVID-19 patients. Key features related to such condition include minimal or no risk of bleeding, moderate thrombocytopenia, high plasma fibrinogen as well as complement components level in the areas of thrombotic microangiopathy. The clinical picture is not typical for classic DIC. This review systematizes the pathogenetic mechanisms of hypercoagulation in sepsis and its extreme forms in patients with COVID-19. The latter consist of the thrombosis-related immune mechanisms, the complement activation, the macrophage activation syndrome, the formation of antiphospholipid antibodies, the hyperferritinemia, and the dysregulation of the renin-angiotensin system. Taking into consideration the pathogenetic mechanisms, the biomarkers had been identified related to the prognosis of the disease development. Patients with pre-existing cardiovascular disease and other risk factors, including obesity, diabetes, hypertension, and aging pose the peak risk of dying from COVID-19. We also summarize new data on platelet and endothelial dysfunction, immunothrombosis, and, as a result, thrombotic storm as essential components of COVID-19 severe features.

Purpose: Cerebral vein thrombosis is a rare, life-threatening condition that has now become more commonly diagnosed due to advancements in imaging techniques. Our purpose is to improve understanding of pathogenesis, diagnosis and pregnancy and IVF management in patients with a history of cerebral thrombosis. Materials and methods: We present an overview of the modern tactics of anticoagulant therapy for cerebral thrombosis with a focus on pregnancy, the use of hormone therapy, and assisted reproductive technologies. Results: The most common risk factors for cerebral vein thrombosis are pregnancy, the postpartum period, and the use of oral contraceptives, which explains the high incidence of this pathology in women. The development of cerebral thrombosis is a vivid example of the interaction and synergetic effects of persistent factors that cause an increased risk of thrombotic complications, which include thrombophilia and acquired risk factors. Despite the possible risks, pregnancy after previously suffered cerebral thrombosis is not contraindicated provided adequate anticoagulant therapy. Conclusions: The most common provoking factors for the development of cerebral thrombosis in women are pregnancy and the use of estrogen-containing drugs. The issue of thromboprophylaxis during pregnancy, when using ART methods and the possibility of using hormonal therapy after cerebral vein thrombosis requires further study.

This article summarizes numerous studies on the relationship of biological processes such as inflammation and thrombosis. The huge role of neutrophils and the extracellular neutrophil traps (NETs) secreted by them has been demonstrated. The discovery of NETs has opened new horizons in the understanding of neutrophil biology and the role of these cells in the body. The use of chromatin in combination with the intracellular proteins, as an effective antimicrobial agent has ancient roots and changes our understanding of chromatin only as a carrier of genetic information. Through NETs, neutrophils can contribute to the development of pathological venous and arterial thrombosis or immunothrombosis, as well as atherosclerosis. NETs release has been shown to be one of the causes of thrombosis in conditions such as sepsis and cancer. The presence of NETs in these diseases and conditions makes it possible to use them or individual components as potential biomarkers. NETs and their components may be attractive as therapeutic targets. Further studies of neutrophils and NETs are needed to develop new approaches to the diagnosis and treatment of inflammatory and thrombotic conditions. Perhaps long-forgotten drugs will find a new area for effective use.

The pandemic of a new coronavirus infection (Coronavirus Disease 2019, COVID-19) caused by SARS-CoV-2 became a real challenge to humanity and the medical community in 2020 and raised a number of medical, social and even philosophical questions. An almost avalanche-like increase in the number of infected people in a short time, due to the high contagiousness of viral infection, allowed us to identify groups of patients with mild, moderate and severe forms of the disease. Doctors around the world are faced with an acute problem of treating a large number of patients in critical conditions caused by COVID-19. From the currently available information on clinical cases of COVID-19, it follows that COVID-19 patients in critical condition have a clinical picture of disseminated intravascular coagulation (DIC), septic shock with the development of multiple organ failure. The first part of the article discusses the pathogenesis of non-specific universal biological responses of the body in critical condition - from the Sanarelli-Schwartzman phenomenon to the DIC, septic shock, systemic inflammatory response syndrome and the so-called neutrophil extracellular traps (NETs). The questions of cytokine storm in severe forms of systemic inflammatory response syndrome (SIRS), the role of inflammation in the activation of coagulation, and the relationship between inflammation and thrombosis are discussed. Modern ideas about the mechanisms of so-called NETosis, their role in the occurrence of immunothrombosis and inflammation-induced thrombosis in autoimmune diseases - vasculitis, antiphospholipid syndrome, and systemic lupus erythematosus is highlighted. The article discusses the possibility of participation of ADAMTS-13 metalloproteinase in the pathogenesis of multiple organ failure in severe endotheliopathy in patients with viral septic shock.