Abstract

INTRODUCTION: Transjugular intrahepatic portosystemic shunt (TIPS) procedures are performed to decrease the complications of portal hypertension in cirrhosis including salvage for variceal bleeds, medically refractory ascites, and hepatic hydrothorax. Although generally well-tolerated, encephalopathy and liver failure are major complications which can occur after the procedure. One of the lesser known complications of TIPS, however, is a transient hemolytic anemia, which we discuss here. It occurs in approximately 10% of cases. CASE DESCRIPTION/METHODS: A 65-year-old male with history of alcoholic cirrhosis decompensated by esophageal and gastric varices was referred to our facility after recent gastrointestinal hemorrhage to evaluate for a TIPS procedure. After being deemed a candidate, a 90 mm total length (70 mm covered / 20 mm uncovered) Viatorr (GORE, Flagstaff, AZ, USA) polytetrafluoroethylene (PFTE) stent-graft was successfully placed, and the portosystemic gradient decreased from 16 mmHg to 5 mmHg. Post-procedure, however, the patient developed worsening jaundice. Laboratory data demonstrated anemia with hemoglobin of 6.0 gm/dL, reticulocytosis with a reticulocyte index of 7.9%, hyperbilirubinemia with up-trending total bilirubin from 6.5 mg/dL to 18.9 mg/dL and an indirect bilirubin of 8.0 mg/dL, and findings consistent with hemolysis with an elevated LDH of 254 units/L, low haptoglobin of < 15 mg/dL, and a negative Coomb’s test. Peripheral smear showed anisocytosis, Burr cells, and target cells; no schistocytes were observed. This led to a diagnosis of non-immune hemolytic anemia. During the remainder of the patient’s hospital course, his hyperbilirubinemia slowly improved prior to discharge. DISCUSSION: Hemolytic anemia is one of the lesser known complications following TIPS. It was first described in the 1990s when bare metal stents (BMS) were commonly used. Literature refers to the hemolytic process with BMS as the “Naked Stent” syndrome due to injury of red blood cells after colliding with the exposed mesh of the stent. In order to improve stent patency and decrease complications, PFTE stents were introduced; however, hemolytic anemia may still occur as described in our case. The pathogenesis remains the same due to stent-related trauma to red blood cells as the covered PFTE stents have areas which remain exposed. With complete endotheliazation of the stent, the hemolytic anemia generally resolves without further intervention in 8–12 weeks.

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