When hemolysis overwhelms the liver: warm autoimmune hemolytic anemia complicated by secondary cholestasis and pigment choledocholithiasis.

INTRODUCTION

Hemolysis is a physiologic condition that results from the lysis of red blood cells and the release of their contents into the plasma. This condition may occur in the setting of a genetic, infectious, mechanical, or immune-mediated process. Classically, hemolysis results in a primarily indirect hyperbilirubinemia as free hemoglobin is metabolized to bilirubin, which is then conjugated and excreted; however, in rare instances, severe and chronic hemolysis may saturate the biliary excretion system and result in a direct hyperbilirubinemia.

METHODS

In this report, we present a 73-year-old man who presented with severe jaundice and was found to have warm autoimmune hemolytic anemia.

RESULTS

The patient's total bilirubin level was 72.4 mg/dL, with a direct bilirubin level of 43.6 mg/dL and elevated liver enzymes. Imaging was consistent with an obstructive process, and hilar thickening on magnetic resonance cholangiopancreatography raised concern for a superimposed cholangiocarcinoma. Endoscopic retrograde cholangiopancreatography, however, removed several black pigment stones, known to be associated with chronic hemolysis, and cytology brushings were negative for malignancy.

DISCUSSION: This case is an excellent illustration of how sustained extravascular hemolysis can overwhelm hepatic excretory capacity and produce secondary cholestasis that mimics malignancy.