Abstract: PUB465
Role of Eculizumab in Postpartum Atypical Hemolytic Syndrome in a Kidney Transplant Recipient
Session Information
Category: Trainee Case Reports
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Awan, Hassan M., UTHSC, Memphis, Tennessee, United States
- Talwar, Manish, UTHSC, Memphis, Tennessee, United States
- Dasari, Aravind, UTHSC, Memphis, Tennessee, United States
- Rajan, Sandeep K., UTHSC, Memphis, Tennessee, United States
- Wall, Barry M., Veterans Affairs Medical Center, Memphis, Tennessee, United States
Introduction
Pregnancy-associated atypical hemolytic uremic syndrome (aHUS) is a rare, potentially lethal condition that can complicate pregnancy in upto 1 in 25000 cases. Given the broad spectrum of conditions that can present similarly in the peripartum period, it is difficult to establish the correct diagnosis in a timely manner. The C5 complement cascade inhibitor eculizumab has been used with considerable success in non-pregnancy aHUS; however, its use in pregnancy associated aHUS is limited to isolated case reports. We present a case of pregnancy associated aHUS in a kidney transplant recipient that was successfully treated with eculizumab with recovery of renal function and safe reintroduction of transplant medications.
Case Description
33 year old female with history of end stage renal disease due to IgA nephropathy who received a cadaveric kidney transplant 7 years prior, presented at 25 weeks gestation with severe hypertension and acute kidney injury. Immunosuppression included tacrolimus, azathioprine and prednisone. Her baseline Cr was <1.0 mg/dl and was 4.7 mg/dl at presentation. She had thrombocytopenia (58 K/ul, down from baseline normal) and hemolytic anemia with high LDH of 1810 U/L, depleted haptoglobin (< 8mg/dl) and presence of schistocytes. She underwent cesarean section for suspected pre-eclampsia with a non-viable fetus. She remained hypertensive, anemic with high LDH, thrombocytopenic, and had worsened renal function with Cr of 7.7 mg/dl. Schistocytes persisted and ADAMTS13 was normal. Tacrolimus was held with concern of drug induced thrombotic microangiopathy (TMA); however, no improvement occurred. Kidney biopsy, initially avoided due to low platelets, was done at week 4 and confirmed the presence of TMA with no evidence of rejection.
Discussion
After exclusion of other etiologies, a diagnosis of aHUS was made and eculizumab initiated. Patient had rapid improvement in renal function by day 4 of induction (Cr of 3.5 mg/dl), platelets increased to 255 K/ul, and LDH decreased to 200unit/L. While on consolidative phase of eculizumab, azathiorine and prednisone were continued. Tacrolimanus was also safely restarted and subsequently switched to belatacept, which she has been tolerating well. This case provides additional support for the role of eculizumab in the treatment of life threatening post-partum aHUS.