RATIONALE OF HEMOCOAGULATION FEATURES IN CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME AND THEIR CONSEQUENCES

Kuzmina G.P.1, Mudra L.O.2, Khаkіmоvа Т.V.2

Summary. Antiphospholipid syndrome (APS) is an autoimmune thrombo-inflammatory disease characterized by a persistent elevation of lupus anticoagulant, anti-β2-glycoprotein-1 (β2GPI), and anti-cardiolipin antibodies in the patient’s blood. Catastrophic antiphospholipid syndrome (CAPS) is a rare and potentially lethal form of APS, characterized by severe thrombotic complications occurring in multiple organs within a short period or simultaneously. The complement-mediated mechanism in the genesis of this thrombo-inflammatory disease is gaining relevance. By activating neutrophils through the formation of DNA extrusions (neutrophil extracellular traps), which possess pro-inflammatory and prothrombotic characteristics, the complement system influences the development of a thrombotic state. CAPS can cause venous, arterial, or microvascular thrombosis in the deep veins of the lower extremities, as well as in the cerebral arterial circulation. Thrombosis can also occur in the hepatic or visceral veins, or within the cerebral venous circulation. The aim of this study is to analyze the disorders of coagulation hemostasis associated with various CAPS phenotypes.

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