Rescue therapy with eculizumab in a transplant recipient with atypical haemolytic-uraemic syndrome

Eculizumab in atypical haemolytic–uraemic syndrome allows cessation of plasma exchange and dialysis

Disorders in complement regulation are a major cause of atypical haemolytic-uraemic syndrome (aHUS). Eculizumab, a monoclonal antibody targeting complement C5 and blocking the terminal complement cascade, should theoretically be useful in this disease, particularly when associated with specific complement pathway anomalies such as Factor H deficiency. Eculizumab is emerging as an effective trea...

Early treatment with eculizumab in atypical haemolytic uraemic syndrome

Atypical haemolytic uraemic syndrome (aHUS) is a rare and life-threatening disease caused by complement system dysregulation leading to uncontrolled complement activation and thrombotic microangiopathy. We report the case of an adult patient with plasmaphaeresis-resistant aHUS and hypertension treated with the complement inhibitor eculizumab. Eculizumab was shown to completely inhibit haemolysi...

Protecting the kidney from complement: atypical haemolytic uraemic syndrome.

Atypical haemolytic uraemic syndrome is a thrombotic microangiopathy with a predominant renal phenotype. Research developments in the last 15 years have led to the elucidation of the role of complement over activation in the pathogenesis of the disease. This was to lead to the successful introduction of targeted pharmacological therapy, in the form of the complement inhibitor, eculizumab.

How I treat thrombotic thrombocytopenic purpura and atypical haemolytic uraemic syndrome

Thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS) are acute, rare life-threatening thrombotic microangiopathies that require rapid diagnosis and treatment. They are defined by microangiopathic haemolytic anaemia and thrombocytopenia, with renal involvement primarily in aHUS and neurological and cardiological sequelae in TTP. Prompt treatment for most case...

Anti-complement C5 therapy with eculizumab for the treatment of Paroxysmal Nocturnal Haemoglobinuria and Atypical Haemolytic Uraemic Syndrome