1887
Volume 2005, Issue 2
  • ISSN: 0253-8253
  • E-ISSN: 2227-0426

Abstract

Hemolytic uraemic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP) are described as acute syndromes with multisystem abnormalities and pentad of thrombocytopenia, microangiopathic hemolysis, neurological symptoms, renal impairment and fever. Both 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 wilbrand factor (vWF) cleavage protease. When renal involvement is severe with little or no neurological manifestation, this microangiopathy is termed as haemolytic-uraemic syndrome. If the hemolytic uraemic syndrome is not associated with diarrhoea, it is called Dnegative 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 differs from syndrome of hemolysis with elevated liver enzymes (HE LLP syndrome) and acute fatty liver of pregnancy hence accurate diagnosis is important for optimal therapy. Plasma transfusion and plasmapheresis have revolutionized management of TTP and HUS by increasing survival 80% to 90%. Here we are reporting a case of D-negative hemolytic uraemic syndrome associated with pregnancy causing intrauterine fetal death. Diagnosis made on clinical and hematological findings, successfully treated by plasmapheresis with residual maternal renal impairment. We are presenting this case, as it is rare disorder associated with high mortality and morbidity, to increase awareness about disease, its diagnosis and management.

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2005-11-01
2019-12-14
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