Acute Hyperoxaluria in Setting of Hereditary Spherocytosis Presenting as a Rapidly Progressive Glomerulonephritis (RPGN)
- AKI: Mechanisms - Case Reports
November 03, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Acute Kidney Injury
- 103 AKI: Mechanisms
- Mcmullan, Barbara C., Vanderbilt University Medical Center, Nashville, Tennessee, United States
- Burgner, Anna Marie, Vanderbilt University Medical Center, Nashville, Tennessee, United States
Hereditary spherocytosis (HS) is the most common inherited hemolytic anemia. Characteristic symptoms include anemia, jaundice, and splenomegaly. Clinical severity is variable with most remaining well compensated. Common complications include cholelithiasis, hemolytic episodes, and aplastic crisis. Despite causing hemolytic anemia, kidney injury is rarely associated with this disease.
A 32-year-old female with HS developed hemolytic crisis and AKI and was aggressively resuscitated. She had evidence of a rapidly progressive glomerulonephritis (RPGN) by labs, see Table 1. She was treated empirically with steroids and underwent kidney biopsy on hospital day 5. Biopsy results showed diffuse calcium oxalate crystals with minimal glomerular injury, see figure 1. Day 1 post biopsy she required dialysis for metabolic derangements. With normalization of electrolytes and clearance of oxalate, she was able to cease dialysis with excellent kidney recovery.
The source of acute oxalosis was unknown. Erythrocytes carry oxalate but intact red blood cells do not increase risk of crystal formation. Red cell membrane fragments have been shown to promote oxalate crystal growth and aggregation up to 2.5 fold. However, she had a history of prior hemolytic episodes without AKIs or nephrolithiasis.
We ruled out causes of oxalate including history of bariatric surgery and ethylene glycol poisoning. We learned that 2 days prior to admission she had received an infusion containing 2500mg vitamin C (Myers cocktail). We postulate that hypervitaminosis C in setting of hemolytic anemia caused acute oxalate crystal deposition with subsequent kidney failure. Various infusions have become readily available with minimal medical oversight, thus history taking remains paramount to patient care.
Red = RRT day
Diffuse tubular injury with oxalate crystal deposition and focal hemosiderin tubulopathy