Plasma Exchange for Hemolytic Crisis and Acute Liver Failure in Wilson Disease

Abstract

Wilson disease (WD) is a rare autosomal recessive disorder of copper metabolism which primarily involves the liver and the central nervous system. Rarely, WD can present as acute liver failure (ALF) and this disease is universally fatal in the absence of liver transplantation. The authors report a young girl with WD ALF, who showed signs of recovery after prompt initiation of plasma exchange (PE) and chelation therapy. Though liver transplantation could not be done in this child and the child died 8 d after stopping PE, this case highlights that PE can be a successful medical treatment in WD ALF and should be considered as a therapeutic measure to stabilize a patient by decreasing serum copper, reducing hemolysis, and helping to prevent renal tubular injury from copper and copper complexes until liver transplantation is possible.

Single Pass Albumin Dialysis and Plasma Exchange for Copper Toxicity in Acute Wilson Disease

“…An added advantage in this clinical situation is the replenishment of coagulation factors with plasma replacement. The efficacy of PLEX in removing excess copper in patients with fulminant WD has been illustrated in several case reports [6, 10, 12, 13, 18-21]. In our second patient, serum copper levels were reduced by 47% after the first PLEX, confirming its efficacy.…”

Plasma Exchange for Hemolytic Crisis and Acute Liver Failure in Wilson Disease: Authors’ Reply

“…To the Editor: We thank Dr Kumar for taking interest in our article [1]. We agree with the author that use of modalities like plasmapheresis should be considered in Wilson's disease-Acute liver failure (WD-ALF); as seen in our case and in other cases mentioned by the author, plasmapheresis can either provide a bridge to liver transplant or rarely prevent the transplant.…”

Evaluation of the pediatric patient for liver transplantation: 2014 practice guideline by the american association for the study of liver diseases, american society of transplantation and the north american society for pediatric gastroenterology, hepatolo

“…Wilson's disease (WD) is a chronic liver condition with a myriad of presentations. WD may be clinically indistinguishable from autoimmune hepatitis, nonalcoholic fatty liver disease, or cryptogenic cirrhosis . The most dramatic presentation is fulminant WD, particularly when encephalopathy is present.…”

“…WD presenting with an acute hemolytic crisis carries a poor prognosis; short‐term clinical and biochemical improvement following plasma exchange coupled with chelation therapy is noted, but outcomes are variable . The AASLD produced joint adult and pediatric guidelines that include recommendations for liver transplant evaluation …”