Kidney Week

Abstract: PUB462

Atypical Hemolytic Uremic Syndrome Overlapping with Antiphospholipid Antibody Syndrome: A Case Report with Four Years Follow Up

Session Information

• Publication Only

Category: Trainee Case Reports

• 102 AKI: Clinical, Outcomes, and Trials

Authors

• Adomako, Emmanuel A., Englewood Hospital and Medical Center, Englewood, New Jersey, United States

Emmanuel A. Adomako,

• Edo-Osagie, Eseosa, Englewood Hospital and Medical Center, Englewood, New Jersey, United States

Eseosa Edo-Osagie,

• Leibowitz, Evan, Prospect Medical Offices Valley Medical Group, Midland PArk, New Jersey, United States

Evan Leibowitz,

Introduction

Overlapping presentation of atypical hemolytic uremic syndrome (aHUS) and antiphospholipid antibody syndrome (APS) is a rare phenomenon. We present a case with four year follow up .

Case Description

A 57-year-old Caucasian female with a history of APS first diagnosed 10 years prior, presented with a year’s history of recurrent abdominal pain and cognitive dysfunction. Laboratory evaluation showed evidence of microangiopathic hemolytic anemia with schistocytes, significant thrombocytopenia and renal insufficiency. Her laboratory studies are summarized below in Table 1. MRI of the brain showed chronic cerebellar and cerebral infarcts. She underwent plasmapheresis for one week without significant improvement in her symptoms or laboratory abnormalities. Given the presentation of thrombotic microangiopathy, the ADAMTS13 activity was > 5%, significant renal insufficiency and the lack of significant response to plasmapheresis, a diagnosis of aHUS was made. She was started on eculizumab infusions, initially 900mg weekly and subsequently maintained on 1200mg every two weeks. After 2 weeks of treatment, her creatinine decreased to 1.2 and hemoglobin stabilized at 9.8g/dl. She has had consistent follow up for 4 years during which period her creatinine has remained stable without the need for renal replacement therapy.

Discussion

aHUS is a condition associated with alternative pathway complement dysregulation. It is characterized by thrombotic microangiopathy(TMA), thrombocytopenia and end organ damage. Clinical presentation of aHUS is difficult to distinguish from other causes of TMA and laboratory differentiation does not always have a quick turnaround time. With an incidence of 1-2 per million, diagnosing aHUS requires a high index of suspicion and the exclusion of thrombotic thrombocytopenic purpura (TTP). APS is a recognized cause of secondary aHUS but may mask its presentation. Timely recognition of aHUS in such cases and the initiation of eculizumab is essential towards modulation of the course of the disease including onset of ESRD and dialysis dependence.

Table 1:Laboratory parameters at presentation and at four-year follow up