Identification and management of acquired von Willebrand disease during extracorporeal membrane oxygenation support
Hemophilia encompasses a group of hereditary bleeding disorders characterized by impaired clotting factor activity,leading to prolonged clotting times.Patients display a tendency toward clotting issues following minor injuries,and severe cases may experience spontaneous bleeding.Acquired von Willebrand syndrome(AvWS)occurs due to the reduction of von Willebrand factor(vWF)levels,resulting in impaired platelet adhesion to endothelial cells,thereby compromising clotting function and leading to bleeding events.The increasing use of extracorporeal membrane oxygenation(ECMO)in clinical settings has brought attention to ECMO-related AvWS.During ECMO support,patients'blood exposure to high shear forces and non-physiological conditions can exacerbate the reduction of vWF levels,further impacting coagulation function.The precise mechanisms triggering AvWS during ECMO support are not conclusively defined,however,studies indicate that high shear forces and systemic inflammation response syndrome(SIRS)are key factors.Mechanical shear stress induced by ECMO damages endothelial cells,releasing factors associated with von Willebrand disease(vWD).Additionally,ECMO-induced SIRS may further compromise vWF functionality.Understanding these mechanisms is crucial for formulating effective preventive and treatment strategies.Diagnosing AvWS during ECMO support can be complex.Typically,assessing a patient's coagulation function and related factor levels is necessary,while cautious interpretation is vital due to potential ECMO interferences.Treatment strategies for managing AvWS during ECMO support are still under investigation.Some studies suggest that using plasma products may improve coagulation function.However,specific treatment approaches should be tailored to individual patient conditions and adjusted based on close monitoring.In summary,diagnosing and treating AvWS during ECMO support remains complex and challenging.Further research holds promise for better understanding the mechanisms involved and for developing more effective treatment strategies to enhance patient prognosis and quality of life.
Extracorporeal membrane oxygenationvon Willebrand factorAcquired von Willebrand syndrome
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