ABSTRACT 239

Abstract

Background and aims: Thrombotic microangiopathy includes thrombotic thrombocytopenic purpura (TTP), atypical haemolytic uremic syndrome (aHUS) and microangiopathies from pregnancy. Due to the high morbidity and mortality an early diagnosis and treatment are important. Aims: Describes a case of aHUS postpartum in a 14-year old patient. Methods: Reported case Results: The patient was admitted to UCIP on day 5 post delivery of 35.6 weeks pregnancy with puerperal sepsis, secondary septic shock, acute renal failure, thrombocytopenia, hemolytic anemia and high LDH (value 1194). Diagnosis of Thrombotic microangiopathy aHUS vs TTP. She requires daily plasma exchange (6 days) to platelet recovery. CVVH (9 days) and peritoneal dialysis (19 days) achieving creatinine normalization and recovery of diuresis. Released after 38 days of hospitalization. aHUS is confirmed with report of ADAMST - 13 > 5%. Chronic treatment with eculizumab is started, prior receiving meningococcal vaccine. Discussion: Thrombotic microangiopathy, in postpartum, requires early management. When assessing the clinical response to plasma exchange and upon no improvement in the values??of LDH, platelets and creatinine or clinical picture recurrence when suspended, you should think of aHUS, request ADAMTS-13 and initiate chronic management with eculizumab. Conclusions: The aHUS associated with pregnancy occurs with long-term maternal morbidity, including renal failure and hypertension. The eculizumab is a suitable alternative to improve the poor prognosis in patients with aHUS post delivery.