Kidney Week

Abstract: SA-PO066

Thrombotic Microangiopathy Treated with Eculizumab in a Patient with Known Immune Thrombocytopenic Purpura

Session Information

• AKI: Clinical, Outcomes, and Trials - Management
  October 26, 2024 | Location: Exhibit Hall, Convention Center
  Abstract Time: 10:00 AM - 12:00 PM

Category: Acute Kidney Injury

• 102 AKI: Clinical, Outcomes, and Trials

Authors

• Koksal, Ali Riza, Banner - University Medical Center Tucson, Tucson, Arizona, United States

Ali Riza Koksal, MD

• Vahdani, Golnaz, Banner - University Medical Center Tucson, Tucson, Arizona, United States

Golnaz Vahdani, MD, MPH

• Ozpolat, Hasan Tahsin, Banner - University Medical Center Tucson, Tucson, Arizona, United States

Hasan Tahsin Ozpolat, MD

• Bracamonte, Erika R., Banner - University Medical Center Tucson, Tucson, Arizona, United States

Erika R. Bracamonte, MD

• Mansour, Iyad S. M., Banner - University Medical Center Tucson, Tucson, Arizona, United States

Iyad S. M. Mansour, MD, MBChB

• Thajudeen, Bijin, Banner - University Medical Center Tucson, Tucson, Arizona, United States

Bijin Thajudeen, MD, FASN

Introduction

Thrombotic Microangiopathy (TMA) is rare but part of a broad spectrum of syndromes that can cause severe kidney failure. Here, we present a TMA case that was previously followed by hematology with a diagnosis of immune thrombocytopenic purpura (ITP), which was proven by a kidney biopsy despite severe thrombocytopenia.

Case Description

A 64-year-old female with traumatic fever, mitral stenosis post mechanical valve replacement, and thrombocytopenia due to ITP presented to ED with severe headache, dizziness, and double vision for 3 days. Cranial CT scan showed subdural hemorrhages, along with trans tentorial herniation. She underwent successful suboccipital craniotomy and and was transferred to the ICU. Hematology was consulted for worsening thrombocytopenia measuring 49 K, down from 102 K on admission, prompting the initiation of 1 mg/kilogram/day prednisone. The patient experienced episodes of hypotension resulting in acute kidney injury. On admission creatinine was 0.7 mg/dL and increased to 4.8 mg/dL in one week. Urine sediment microscopy showed multiple granular casts WBCs and rare isomorphic RBCs, UACR 78 mg, UPCR 800 mg, and urine NGAL:492. Renal ultrasound showed no signs of obstruction or chronicity. Subsequently, her thrombocytopenia continued to worsen along with increasing creatinine, but on peripheral smear there was no evidence of schistocytes, ADAMTS13 activity was normal, and GN panel was negative. She required intermittent HD for clearance and ultrafiltration. Kidney biopsy was performed due to delayed kidney recovery, and revealed thrombotic microangiopathy with predominant chronic lesions, with patchy moderate interstitial fibrosis 40%. Complement- targeted therapy was started with diagnosis of chronic TMA/atypical Hemolytic Uremic Syndrome (HUS) vs. renal limited TMA. She received an induction of Eculizumab 900 mg weekly for one month and continued with maintenance thereafter. The patient creatinine improved, and hemodialysis was discontinued. Long-term creatinine was stable at 2.5 mg/dL on third month follow-up visit.

Discussion

Severely progressive AKI with thrombocytopenia can be related to TMA with a component of atypical HUS. Eculizumab is a good option for maintaining immunosuppression when there is a concern of atypical HUS.