2206 Nodular Regenerative Hyperplasia in a Patient With Sickle Cell Disease

Abstract

INTRODUCTION: Nodular regenerative hyperplasia (NRH) is a rare liver condition caused by transformation of hepatic parenchyma into regenerative nodules. Despite the association between NRH and certain hematologic disorders, specifically myeloproliferative diseases, there are very few reports of NRH associated with sickle cell disease (SCD). We present a patient with SCD with liver biopsy confirmation of NRH and sickle cell hepatopathy. CASE DESCRIPTION/METHODS: A 45-year-old male with history of hypertension and sickle cell anemia (hemoglobin SS phenotype) was initially referred to hepatology due to concerns for cirrhosis on CT scan. He had been admitted for significant fatigue and a CT thorax was obtained due to opacity on chest X-ray. This study showed findings concerning for cirrhosis with high density in the liver suggesting hemosiderosis; this was new compared to imaging five years prior. Upon initial evaluation in clinic, he was asymptomatic and fatigue had resolved since receiving blood transfusion during his hospitalization. He had normal vital signs and benign physical exam. Pertinent laboratory results included: AST 39 IU/L, ALT 19 IU/L, Total Bilirubin 2.35 mg/dL (Direct Bilirubin 0.7 mg/dL), Alkaline Phosphatase 81 IU/L, INR 1.0, Hemoglobin 8.3 gm/dL. His ferritin was 1021 ng/mL, a hepatitis panel was negative, and anti-mitochondrial antibody was elevated at 24.5 units. A liver ultrasound with dopplers demonstrated increased echogenicity, mildly coarse echotexture, with an irregular contour consistent with cirrhosis. To delineate the cause of his liver disease, a percutaneous liver biopsy was obtained. Findings on pathology were consistent with sickle cell hepatopathy with nodular regenerative hyperplasia, mild hepatocellular and Kupffer cell siderosis, and no fibrosis. He is planned for interval follow up with hepatology. DISCUSSION: SCD is associated with several liver complications including infarcts, abscesses, and hemosiderosis, with NRH being an uncommon complication. The proposed mechanism of NRH as it relates to SCD is due to obstructive portal venopathy resulting in areas of compromised blood flow with regenerative nodules forming in other areas of the liver to compensate for this. As it is asymptomatic with a prolonged subclinical period, it is frequently missed, however early recognition is important to avoid complications related to non-cirrhotic portal hypertension. This case highlights a very rare association between NRH and SCD.