Acute Massive Hepatic Enlargement in Sickle Cell Disease

Abstract

Introduction: Enlargement of the liver is a common condition in clinical practice and has multiple etiologies. Acute massive increase in liver size within a matter of 48 hrs is an unusual event. We report a case of a 25-year-old female patient with sickle cell disease who presented with right upper quadrant abdominal pain, fever, and leukocytosis of 36,000 mm3. A urine analysis suggested UTI and she was treated with antibiotics along with intravenous fluids. On the 2nd hospital day, the hemoglobin dropped to 6.8g/dl from 8.2g/dl on admission. The leukocytosis increased to 45,900 mm3 and the total bilirubin from a baseline of 9.0 mg/dl on admission, rose to 12.7 mg/dl two days later. A CT scan of the abdomen on admission showed a liver of normal size with mild intrahepatic biliary ductal dilatation. Two days later, with persistent right upper quadrant discomfort, worsening leukocytosis, and the rapid drop in hemoglobin, the patient underwent a repeat CT scan followed by MRI of the abdomen. In 2 days, the liver size had drastically increased from 17.5 cm to 20.5 cm in vertical length along the mid clavicular line. An infiltrative or cobble stone type pattern had appeared in the liver parenchyma. There was no intrahepatic or common duct dilatation. The hepatic and portal veins were patent. On subsequent vascular duplex study of the inferior vena cava (IVC), the hepatic and portal veins showed no evidence of thrombosis. Because of the patient’s deteriorating status, exchange transfusion was performed, following which, the liver function tests improved and liver size clinically diminished. This intriguing case illustrates the rare syndrome of hepatic sequestration in sickle cell disease. Our patient had an acute increase in liver size, which was not due to hepatic or portal vein thrombosis; nor was it due to cholestasis. Sickle cell disease may result in sequestration of red blood cells in the spleen or lungs. On occasion, the liver is the site of RBC sequestration. In addition to pain and a falling hematocrit, liver size increases rapidly. This sequence of events in the liver may be missed unless liver size is regularly measured. When hepatic sequestration occurs, prognosis is grave and exchange transfusion should be considered as a potentially life saving strategy. Our patient with sickle cell disease had a dramatic picture of an acutely enlarging liver, which with appropriate treatment rapidly returned to normal size.