Researchers from the United States presented the case of a woman with Wilson disease (WD) whose mental status improved following plasmapheresis.
“Plasmapheresis remains an important treatment option for preventing rapid clinical deterioration and worsening complications associated with Wilson’s disease,” the researchers concluded.
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The case study is published in the scientific journal Case Reports in Hepatology.
“It continues to serve as a bridge to liver transplant and may be used for longer periods in certain clinical contexts in association with other therapeutic interventions like chelating agents, as liver transplants may not be readily available or patients may not meet the criteria for an immediate liver transplant,” they added.
The woman’s condition was complicated by coagulopathy, acute kidney injury, impaired liver function, and hemolysis. She also reported lethargy.
Plasmapheresis was used as a bridge to a liver transplant and was administered daily from the second day of her hospital admission for 5 days until the transplant.
The treatment improved not only her mental status including sensorium, but also her kidney function, bilirubin levels, international normalized ratio, which is a measure of the time it takes for the blood to clot, and serum creatinine levels.
The researchers from the University of Wisconsin Hospital in Madison, Wisconsin, reported that she subsequently underwent a liver transplant, which was successful and that she remained stable post-transplant.
WD is a rare genetic disease that affects copper metabolism. The disease is characterized by copper accumulation in different tissues and organs, especially the liver, brain, kidneys, and eyes, causing damage.
Symptoms include jaundice, fatigue, loss of appetite, ascites, tremor, ataxia, hypersalivation, hypomimia, dysarthria, clumsiness, Kayser-Fleischer rings, hematuria, arrhythmias, and hemolysis.
Treatment consists of lifelong use of chelating agents such as penicillamine or trientine, but these may exacerbate neurological symptoms when first started. Liver transplant is a curative treatment for the disease.
Reference
Glover Q, Rose WN. Plasmapheresis for fulminant Wilson’s disease improves mental status and coagulopathy. Case Reports Hepatol. Published online June 19, 2023. doi:10.1155/2023/3985823
