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Kidney Week

Abstract: PO2230

Efficacy of Eculizumab Therapy in Delayed-Diagnosed, Hemodialysis-Dependent, Pregnancy-Triggered, Complement-Mediated Thrombotic Microangiopathy

Session Information

Category: Women’s Health and Kidney Diseases

  • 2000 Women’s Health and Kidney Diseases

Authors

  • Huang, Gaoyuan, New York City Health and Hospitals Coney Island, Brooklyn, New York, United States
  • Arister, Nathan Anatoly, New York City Health and Hospitals Coney Island, Brooklyn, New York, United States
  • Bayewitz, Ashrei, New York City Health and Hospitals Coney Island, Brooklyn, New York, United States
  • El-Hennawy, Adel S., New York City Health and Hospitals Coney Island, Brooklyn, New York, United States
  • Okoye, Chibuzo C., New York City Health and Hospitals Coney Island, Brooklyn, New York, United States
  • Frolova, Elena, New York City Health and Hospitals Coney Island, Brooklyn, New York, United States
Introduction

Pregnancy associated atypical hemolitic uremic syndrome (p-aHUS) is provoked by pregnancy and affects 1/25,000 pregnancies in the general population. The delivery is vital for halting the disease process. The course of complement-mediated thrombotic microangiopathy(C-TMA) is not affected by delivery, but condition improves with anti-complement therapy. We are presenting a rare case of delayed diagnosed, pregnancy provoked C-TMA with significant improvement in blood pressure, stabilization of anemia, and resolution of thrombocytopenia after treatment with Eculizumab.

Case Description


A 25-year-old Hispanic woman with history of CKD stage IIIB presented with syncope. She developed renal failure, required hemodialysis(HD) and HTN during her first pregnancy. During postpartum, HD was stopped and HTN resolved. Two years later she was admitted with worsened renal function, severe anemia (Hb 4 g/dl), thrombocytopenia (36K/ul), and poor controlled HTN, needing 6 different classes of drugs to control her blood pressure. Blood smear showed schistocytes. Our extensive work up ruled out: TTP, HUS, DIC, lupus, scleroderma, and other disorders. Complement 3 was low. Renal ultrasound excluded post-renal obstruction and renal artery stenosis, but showed echogenic kidneys. Given echogenic kidneys and increased bleeding risk, renal biopsy was not performed. The diagnosis of C-TMA was established. Regular HD was resumed. She was started on Eculizumab and with maintenance treatment her HTN became well controlled with only two medications.

Discussion

Timely diagnosis and management are the key points to improve C-TMA prognosis. It may be a difficult diagnosis and mimic eclampsia, HELLP syndrome, or p-aHUS. Although it is related to inherited defects of complement alternative pathway or the proteins that regulate it, lack of linked gene mutations cannot exclude C-TMA. In this patient, the diagnosis of C-TMA was not made until two years after onset. Our case report showed that even though Eculizumab cannot completely reverse the renal injury at the late stage of C-TMA, it may still improve the blood pressure control, normalize platelets, help anemia, and prevent further injury.