Background: Hemolytic uremic syndrome (HUS) is a thrombotic microangiopathy defined by the sudden onset of hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI). HUS is categorized as either typical, caused by Shiga toxin-producing Escherichia coli infection, or atypical HUS (aHUS), usually complement mediated or secondary to systemic disease. We describe a rare case of aHUS in an adult male patient with recurrent acute pancreatitis. A 32-year-old Caucasian male presented to our institution for his third episode of alcohol-induced pancreatitis. He presented with abdominal pain, elevated lipase and pancreatic inflammation on computed tomography consistent with acute pancreatitis. While admitted, he developed sudden onset severe thrombocytopenia, AKI and hemolytic anemia. Peripheral blood smear, haptoglobin and hemoglobin level confirmed microangiopathic hemolytic anemia. Worsening anemia, thrombocytopenia and AKI were consistent with the diagnosis of aHUS. The patient's pancreatitis resolved with supportive measures, but resolution of significant thrombocytopenia and AKI was not achieved until administration of eculizumab, a complement inhibiting therapy. Eculizumab therapy provided dramatic improvement in this patient, with platelet count increasing from a low of 11,000 to >100,000 within 48 hours of therapy. Creatinine and hemoglobin levels returned to baseline within 3 weeks.

Conclusions: Recurrent pancreatitis is suggested as the etiology of atypical HUS in this patient and this condition should be recognized and treated in a timely manner for optimal clinical outcomes.