Clinical features of hemolysis, elevated liver enzymes, and low platelet count syndrome in undiagnosed Wilson disease: report of two cases.

INTRODUCTION

Wilson's disease (WD) is an autosomal recessive disorder characterized by toxic accumulation of copper mainly in the liver and brain. The hepatic manifestation of WD is diverse and may include asymptomatic elevation of aminotransferase, chronic hepatitis, cirrhosis, or acute/fulminant hepatic failure. Characteristic of acute hepatic failure in WD is concomitance of acute intravascular hemolytic anemia that in some patients may represent a first clinical symptom of WD. The diagnosis of acute Wilsonian liver failure is difficult, as similar signs may be observed in other clinical conditions. In pregnant patients with unrecognized WD, liver failure with hemolysis may be interpreted as the low platelet count (HELLP) syndrome.

PATIENTS: We describe two women, who developed the clinical features of hemolysis, elevated liver enzymes, and HELLP syndrome. In both, further diagnostics confirmed WD.

CONCLUSION

WD should be remembered in the differential diagnostics of HELLP syndrome.