Original Article - Current Pediatric Research (2025) Volume 29, Issue 1

Plasma exchange as a treatment for hemolytic crisis and acute liver failure in Wilson disease.

Wilson Disease (WD) is a genetic disorder resulting in copper accumulation, leading to liver failure and neurological symptoms. Acute Liver Failure (ALF) in WD often necessitates urgent liver transplantation due to its poor prognosis. This report details the use of Plasma Exchange (PE) in a 7- year-old girl with WD who presented with ALF and hemolytic anemia. Despite the severe clinical condition and laboratory findings indicative of WD, the family opted for PE against immediate liver transplantation. The child was started on PE with the Spectra Optia® Apheresis System and Dpenicillamine and zinc were introduced. Four PE sessions were performed on alternate day, each lasting 1.5 to 2 hours, exchanging approximately 1,100 mL of plasma per session. Post-treatment, the child showed significant improvement in hemolysis, bilirubin levels and liver function tests, providing a temporary stabilization that allowed for bridging to potential transplantation. This case underscores the role of PE as a viable option for managing WD-related ALF, highlighting its effectiveness in reducing copper levels and stabilizing the patient’s condition when transplantation is not immediately feasible. This experience contributes to the growing body of evidence supporting PE as a bridging therapy in severe Wilson disease cases.

Author(s):

Devang Gandhi*, Suraj Patel, Hiteshee Patel

Abstract PDF