scholarly journals Post-partum atypical haemolytic-uraemic syndrome treated with eculizumab: terminal complement activity assessment in clinical practice

2013 ◽  
Vol 6 (4) ◽  
pp. 454-454
Author(s):  
Yahsou Delmas ◽  
Cécile Bordes ◽  
Chantal Loirat ◽  
Véronique Frémeaux-Bacchi ◽  
Christian Combe
2021 ◽  
pp. 1753495X2110199
Author(s):  
Mehmet Nuri Duran ◽  
Fatma Beyazit ◽  
Mesut Erbaş ◽  
Onur Özkavak ◽  
Celal Acar ◽  
...  

Pregnancy‐associated atypical haemolytic uraemic syndrome is a rare and potentially lethal complement-mediated disorder. It can mimic preeclampsia, gestational hypertension, thrombotic thrombocytopenic purpura and hemolysis, elevated liver enzymes and low platelets syndrome. Thus, it can be hard to distinguish pregnancy‐associated atypical haemolytic uraemic syndrome from other causes in peri/post-partum women presenting with features of microangiopathic haemolytic anemia, thrombocytopenia and acute kidney injury. We present a case of a 35-year-old woman in her third pregnancy at 32 weeks’ gestation who underwent caesarean section due to fetal distress. She developed severe renal impairment, thrombocytopenia and neurologic symptoms within 24 hours after delivery. A diagnosis of pregnancy‐associated atypical haemolytic uraemic syndrome was provided, and treatment with plasma therapy followed by eculizumab was initiated. A rapid improvement of both clinical and laboratory parameters was observed. This case demonstrates the significance of early initiation of anti-complement therapy to prevent irreversible renal damage and possible death in women with pregnancy‐associated atypical haemolytic uraemic syndrome.


Author(s):  
Matthew C. Pickering ◽  
Jyoti Bakshi

In this chapter we summarize clinically important aspects of complement biology. This information is used to present a comprehensive overview of complement deficiency states and provide a logical basis for understanding complement assays in clinical practice. Hypocomplementaemic urticarial vasculitis syndrome, a condition associated with anti-C1q antibodies and complement consumption, is discussed. We summarize the use of eculizumab, a monoclonal antibody that targets and inhibits complement C5, in the treatment of haemolysis in paroxysmal nocturnal haemoglobinuria and atypical haemolytic uraemic syndrome.


Author(s):  
Matthew C. Pickering ◽  
Jyoti Bakshi

In this chapter we summarize clinically important aspects of complement biology. This information is used to present a comprehensive overview of complement deficiency states and provide a logical basis for understanding complement assays in clinical practice. Hypocomplementaemic urticarial vasculitis syndrome, a condition associated with anti-C1q antibodies and complement consumption, is discussed. We summarize the use of eculizumab, a monoclonal antibody that targets and inhibits complement C5, in the treatment of haemolysis in paroxysmal nocturnal haemoglobinuria and atypical haemolytic uraemic syndrome.


Author(s):  
Matthew C. Pickering ◽  
Jyoti Bakshi

In this chapter we summarize clinically important aspects of complement biology. This information is used to present a comprehensive overview of complement deficiency states and provide a logical basis for understanding complement assays in clinical practice. Hypocomplementaemic urticarial vasculitis syndrome, a condition associated with anti-C1q antibodies and complement consumption, is discussed. We summarize the use of eculizumab, a monoclonal antibody that targets and inhibits complement C5, in the treatment of haemolysis in paroxysmal nocturnal haemoglobinuria and atypical haemolytic uraemic syndrome.


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