Management of Acute Wilsonian Hepatitis with Severe Hemolysis: A Successful Combination of Chelation and MARS Dialysis

Abstract

Wilson’s disease is a rare hereditary disorder of copper metabolism leading to progressive accumulation of copper in several organs including the brain and the liver. Acute liver failure is a relatively rare hepatic manifestation of WD which may require urgent liver transplantation if medical treatment fails. We report here the case of a young woman who presented with classic acute Wilsonian hepatitis complicated by liver and renal failure and a severe hemolysis related to massive nonceruloplasmin bound copper accumulation requiring repeated blood transfusions. The early initiation of a combined treatment including conventional chelation therapy and repeated MARS dialysis sessions allowed a rapid control of hemolysis, a progressive decrease of free copper overload, and clinical recompensation without liver transplantation.

Copper, Iron, Cadmium, and Arsenic, All Generated in the Universe: Elucidating Their Environmental Impact Risk on Human Health Including Clinical Liver Injury

“…In infants before the age of three years Wilson disease is uncommon [60]. As expected, liver injury as one of the key features in Wilson disease received much attention in various publications [115][116][117][118][119][120][121][122]. Among these, liver manifestations were recently summarized [122].…”

“…Details of clinical manifestations were perfectly and comprehensively listed in [ 106 ]: (1) findings related to the liver may include abnormal LTs, chronic active hepatitis, cirrhosis with portal hypertension, and acute liver failure; (2) psychiatric features comprise affect, cognition, and behavioral disorders as well as depression and psychosis; (3) neurologically, tremor, dysarthria, ataxia, nystagmus, writing problems, and dysphagia with pseudohypersalivation prevail; (4) renal tubular dysfunction; (5) Kayser–Fleischer corneal rings to be verified through split-beam examination by an eye specialist; and (6) various findings like cardiomyopathy, cardiac arrhythmias, rhabdomyolysis, osteoporosis, osteomalacia, arthritis, and arthralgia. In addition, Coombs-negative hemolytic anemia is a key feature of Wilson disease with undetectable serum haptoglobin, high serum activities of lactate dehydrogenase, and high reticulocyte counts [ 119 , 120 ].…”

“…Serum ALP activities were described with low levels for unknown reasons [2,123,124]. In Wilson disease serum activities of AST are often much higher as compared with ALT [119,123]. High serum ALT activities up to 800 U/L were commonly found only in younger children with an age range from 4 to 8 years, with low ALT activities in infants with an age <4 years partially due to hemolytic crisis, and higher ALT values in 3 other infants [118].…”

Wilson’s Disease with Acute Hepatic Onset: How to Diagnose and Treat It

“…Based on some cases [128][129][130] and WD clinical practice guidelines [23], MARS dialysis can stabilize the patient's condition and can act as a bridge to liver transplantation. Reducing the copper load by MARS can confer a benefit in the management of acute decompensated WD [131,132], and sometimes several sessions of MARS may be necessary to achieve the therapeutic goal (cases in the literature report up to 18 sessions of MARS) [133].…”