Introduction: Spur Cell Anemia (SCA) is a rare underrecognized non-autoimmune hemolytic anemia occurring in severe liver disease with prognostic implications. Since the 1980s there have been only a few dozen case reports and clinical images concerning SCA. We present a case of a young man managed in the ICU with multiorgan failure and a masked diagnosis of SCA.
Description: A 33-year-old man was found down and presented to the ICU with hematemesis and hemorrhagic shock. He required massive transfusion for bleeding esophageal varices that were later banded and an expanding thigh hematoma. He was diagnosed with alcohol induced liver cirrhosis with a MELD-Na score of 38 and eventually discharged with persistent anemia. He was not a transplant candidate due to recent alcohol use. He returned three weeks later with anasarca and shortness of breath and was diagnosed with hepatorenal syndrome requiring vasopressors. While back in the ICU his hemoglobin declined to 6 g/dL and remained below goal despite transfusion. His continued anemia was thought to be secondary to portal gastropathy and bleeding in his thigh. No active bleeding was found on physical exam or with repeated CT imaging of his hematoma. His LDH and haptoglobin were 653 IU/L and < 1 mg/dL with a total bilirubin of 25.59 mg/dL prompting work up for hemolytic anemia. The peripheral smear demonstrated moderate acanthocytes, which combined with his refractory hemolytic anemia in severe liver disease established a diagnosis of SCA.
Discussion: SCA in alcoholic cirrhosis is shown to be an independent predictor of mortality with median reported survival of 1.9 months. SCA is managed with supportive care and rapid evaluation for liver transplant which is the only known effective therapy. Pentoxifylline and flunarizine have been explored with little success in small studies and case reports. These measures were considered for our patient, but ultimately, he was transferred to a transplant center and underwent a successful liver transplant with resolution of his SCA. Our diagnosis of SCA was delayed by focus on his other pathologies that more commonly cause anemia. This illustrates why common issues such as anemia require a broad differential in the ICU where pathology is severe and complex.
