Abstract: PUB478
Typical Atypical Hemolytic Uremic Syndrome: A Pregnancy-Induced Story
Session Information
Category: Trainee Case Reports
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Babaniji, Oluwadamilola O., Methodist Hospital Dallas, Dallas, Texas, United States
- Collazo-Maldonado, Roberto L., Dallas Nephrology Associates, Dallas, Texas, United States
Introduction
Pregnancy-associated atypical hemolytic uremic syndrome (p-aHUS) is a rare condition that can mimic other clinical conditions in the postpartum period. It is very important for clinicians to promptly recognize and manage this potentially fatal condition to reduce the catastrophic complications that could result if early diagnosis is not made.
Case Description
A 31 year old primigravida African American woman with past medical history of hypertension, asthma and obesity presented to her post-partum follow up clinic 2 weeks after her C-section delivery of a preterm live infant at 29 weeks .Her pregnancy was complicated by premature ROM and chorioamnionitis. She was found to be very hypertensive (220/120 mmHg) with new onset abdominal pain associated with frontal headache and new-onset bilateral lower extremity edema with no fever and normal mental status. She was immediately admitted to the hospital and her initial laboratories showed new onset acute kidney injury (creatinine 2.5mg/dL), new-onset thrombocytopenia (35000), presence of hemolytic anemia with schistocytes on smear, markedly elevated LDH levels (>2000), and undetectable haptoglobin levels. Urinalysis was notable for active sediment with nephrotic range proteinuria and hematuria. Nephrology was consulted and given her classic presentation, she was started on intravenous steroids and hematology was consulted for emergent plasmapheresis the same day of admission. Diagnosis of atypical hemolytic uremic syndrome was confirmed with normal ADAMTS-13 level. Hemodialysis was eventually initiated for worsening kidney function. She was started on Eculizumab 48 hours after being admitted. After 2 doses of Eculizumab, her blood pressure is controlled with normalization of her hemoglobin and platelets and recovery of her kidney function. Patient is currently undergoing genetic workup for identification of inherited complement abnormalities.
Discussion
p-aHUS is a challenging condition that has been known to present in the early postpartum period. It could easily be mistaken for other well-known postpartum complications such as preeclampsia and HELLP syndrome leading to delay in treatment. The bad outcomes of this entity may be mitigated with quick clinical recognition and treatment initiation.