Abstract: PUB019
From Hemoglobinuria to Hemodialysis: A Case of Paroxysmal Nocturnal Hemoglobinuria-Induced AKI
Session Information
Category: Acute Kidney Injury
- 102 AKI: Clinical, Outcomes, and Trials
Authors
- Gupta, Naman, Virginia Commonwealth University School of Medicine, Richmond, Virginia, United States
- Payne, Charles, Virginia Commonwealth University School of Medicine, Richmond, Virginia, United States
- Mangray, Mahendra, Richmond VA Medical Center, Richmond, Virginia, United States
- Muthusamy, Selvaraj, Virginia Commonwealth University School of Medicine, Richmond, Virginia, United States
- Bobba, Sindhura, Richmond VA Medical Center, Richmond, Virginia, United States
Introduction
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematopoietic disorder marked by complement mediated hemolysis, thrombosis, and marrow dysfunction. Anuric acute kidney injury (AKI) in PNH remains an underreported complication. Here we describe a patient with PNH who presented with hemolytic flare complicated by sudden onset anuric AKI with biopsy proven acute tubular injury with pigment deposition in tubules.
Case Description
A 77 year old man with PNH, Coronary artery disease and Diabetes presented with cola-colored urine and chest pain. Initial workup consistent with type 2 MI and negative for PE on CTA. Hematological workup consistent with hemolysis as evidenced by LDH >1000 U/L and haptoglobin <8 mg/dL, requiring blood transfusion. During hospitalization, the patient became anuric and creatinine rose from 1.3 to 8.5 mg/dL requiring dialysis. Renal duplex ultrasound negative for obstruction or thrombosis. Urine microscopy showed only eumorphic red blood cells. Kidney biopsy showed acute tubular injury with significant cytoplasmic pigment deposits in tubular epithelium. Many tubules demonstrated luminal casts consisting of necrotic debris from tubular epithelial cells. Despite being on Eculizumab, the patient remained on dialysis at the time of submission. Current treatment plan is to continue eculizumab.
Discussion
PNH is a rare but serious disorder in which intravascular hemolysis can lead to kidney injury, via hemosiderin-mediated tubular toxicity. While transient hemoglobinuria is common during flares, AKI is not a common presentation. This case highlights the importance of early recognition of PNH associated with severe AKI from pigment nephropathy.
H&E demonstrating acute tubular injury with hemosiderin pigment deposits (brown) in the proximal tubules