Abstract: SA-PO220
Postpartum Associated Atypical Hemolytic Uremic Syndrome
Session Information
- Trainee Case Reports - V
October 27, 2018 | Location: Exhibit Hall, San Diego Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Trainee Case Reports
- 103 AKI: Mechanisms
Authors
- Shivarov, Hristo, Lenox Hill Hospital - Northwell Health System, Secaucus, New Jersey, United States
- DeVita, Maria V., Lenox Hill Hospital- Northwell Health System, New York, New York, United States
Introduction
Pregnancy associated atypical hemolytic uremic syndrome is a rare, life threatening, progressive systemic disease of uncontrolled activity of the alternate complement pathway resulting in systemic thrombotic microangiopathy during pregnancy or postpartum period. The diagnosis of this condition is challenging and can mimic various diseases found during pregnancy and the postpartum.
Case Description
35 year old woman without significant past medical history who presented to our hospital for elective delivery at 37th weeks gestation. During the induction period the labor arrested, the patient became hypertensive to 170/100 mmHg with subsequent fetal bradycardia necessitating a C-section. Immediately postpartum uterine atony with severe hemorrhage was observed. The patient was intubated and was placed on pressor support.Severe anemia was registered with hemoglobin drop to 6.8 from 12.0 g/dl. The hemoglobin level was 6.9 g/dl after transfusion 4 units of PRBC. There were no overt signs of bleeding. Additional studies showed: LDH 1324 u/l, haptolgobin <10 mg/dl, and platelets – 62 from 212 K/uL; schistocytes were noticed on peripheral smear. Deterioration of the renal function was observed over the next 48 hours with anuria, creatinine peaked to 7.02 mg/dl. Hemodialysis was initiated for oliguria, hyperkalemia and fluid overload. Subsequent studies showed decreased level of C3 at 73 mg/dl with normal C4. Differential diagnosis included TTP, severe pre-eclampsia, aHUS, malignant hypertension and ischemic ATN. Plasma exchange was started with iv prednisone 100 mg/daily, ADAMTS13 was 42% excluding TTP. Eculizumab 900 mg/weekly was started 5 days POD. Gradually the hemoglobin level and platelets stabilized. The patient was extubated and the hemolysis resolved.HD treatment was discontinued on POD 19 after significant improvement of the renal function and good control of blood pressure. During the hospital stay she received total of three doses of eculizumab. The patient was discharged with continuation with eculizumab biweekly
Discussion
Diagnosis of pregnancy associated aHUS must be considered early in postpartum period in patient with hemolysis, rapid deterioration of renal function despite the possibility of superimposed acute tubular injury, abnormal complement function and normal ADAMTS13 activity. Early initiation of treatment with plasma exchange and eculizumab has proven its efficacy in postpartum aHUS.