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

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.

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

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.

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