Hemolytic uraemic syndrome associated with pregnancy

Abstract EN

Hemolytic uraemic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are described as acute syndromes with multisystem abnormalities and pentad of thrombocytopenia, microangiopathic hemolysis, neurologi¬cal symptoms, renal impairment and fever.

Doth diseases were believed to form a continuum of the same disease, but recently it was found, that they were having a different pathophysiology, as TTP patients have a deficiency in von wilbrandfactor (vWF) cleavage protease.

When renal involvement is severe with little or no neu¬rological manifestation, this microangiopatliy is termed as haemolytic-uraemic syndrome.

If the hemolytic uraemic syndrome is not associated with diarrhoea, it is called D- negative or atypical HUS.

This subdivision is of etiological and prognostic importance.

TTP-HUS is associated with high maternal and fetal morbidity and mortality.

Treatment of these syndromes dif¬fers from syndrome of hemolysis with elevated liver en¬zymes (HELLP syndrome) and acute fatty liver of preg¬nancy hence accurate diagnosis is important for optimal therapy.

Plasma transfusion and plasmapheresis have revolu¬tionized management of TTP and HUS by increasing sur-¬vival 80 % to 90 %.

Here we are reporting a case of D-negative hemolytic uraemic syndrome associated with pregnancy causing in¬trauterine fetal death.

Diagnosis made on clinical and he¬matological findings, successfully treated by plasmapher¬esis with residual maternal renal impairment.

We are presenting this case, as it is rare disorder asso¬ciated with high mortality and morbidity, to increase aware¬ness about disease, its diagnosis and management.