One complicated case of thrombotic microangiopathy

One complicated case of thrombotic microangiopathy

Author Guliko Kiliptari
Co-Author Merab Sutidze
DOI

Country : Georgia
Subject : critical care

Keywords : HUS,renal replacement therapy,coma,vena cava thrombosis

Abstract

HUS and TTP is syndroms,charactarized with microangiopathic hemolityc anemia, trombocytopenia, acute renal falure,severe neurological violations.Bloody diarrhea is caused with E.Coli(0157:H7).In georgia revealed other strain –E.coli(0104:H4).We prezented case when ilness started with bloody diarrhea, oliguria and neurological changing( coma, seizures.).. ADAMTS13 levels < 10% with the presence of antibody against ADAMTS13 is characteristic of most adults with TTP and these patients respond to plasma exchange. Testing for ADAMTS13 activity is appropriate in patients with suspected TTP-HUS, The combination of clinical and laboratory data, activity of ADAMTS13, and response to plasma exchange allows for better differentiation between these thrombotic microangiopathies, which itself is very important considering that both have different treatment options. Thrombotic microangiopathies are diseases characterized by thrombocytopenia, erythrocyte fragmentation, and elevated levels of LDH. Thickening of the arterioles and capillary walls with prominent endothelial swelling and detachment and subendothelial accumulation of proteins and cell debris characterize and define the pathologic lesion seen in all thrombotic microangiopathies. In patients with TTP, severely deficient ADAMTS13 activity has been seen in 25–79% of cases at presentation, whereas HUS is not associated with any reduction in activity or absence of ADAMTS13. Patient admitted in hospital after one weak from onset of clinical simptoms. Regardless of bacteriological investigations of feces, the microb does not revealed.Progress of disease was severe,with many complication:renal failure with severest neurological violations. Unconsciousness was manifested after hospitalization with generalized seizures.MRI was rivealed temporal and parietal cortex damage,later left ischemic damage of left subcoritical nodes,what probably was the reason of seizures. LDH and haptoglobin level was reffered microangiopathic haemolysis. In the smears of peripheral blood was observed erythrocyte fragmentation.Platelets counts was mildly decreased, FDP increased (D dimer also increased).Therefore genesis of renal failure and coma was thrombotic microangiopathy and other encompanying causes. In this patient, despite such extensive involvement of the CNS, ADAMTS13 activity was not inadequate, the treatment was effective.

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