Vaskulárna medicína 2/2025
Complement-mediated thrombotic microangiopathy emphasizing renal involvement in children
Purpose: Complement-mediated thrombotic microangiopathy is a relatively heterogeneous group of thrombotic microangiopathies. It typically manifests as a life-threatening condition, most commonly associated with kidney injury. The pathophysiology involves dysregulation of the alternative complement pathway, resulting in endothelial cell dysfunction and increased thrombus formation within the microcirculation. Key clinical features include microangiopathic hemolytic anemia and thrombocytopenia, accompanied by organ damage – most often affecting the kidneys. This article aims to provide a comprehensive overview of two pediatric case reports of complement-mediated thrombotic microangiopathy, emphasizing renal involvement.
Case: The presented case studies describe not only the clinical course of the disease but also the therapeutic management and long-term follow-up of the patient’s health status.
Conclusion: The cornerstone of treatment for complement-mediated thrombotic microangiopathy, both in the acute phase and during long-term management, is the administration of humanized monoclonal antibodies (eculizumab or ravulizumab). These agents target the C5 component of the complement cascade, thereby preventing the formation of the membrane attack complex. Their use has been well documented in several comprehensive studies demonstrating the safety and efficacy of this therapeutic approach.
Keywords: trombotická mikroangiopatia, atypický hemolyticko-uremický syndróm, poškodenie obličiek, detský vek
