Acute Hepatic Sequestration in Sickle Cell Disease: Differential Diagnosis
Differential Diagnosis of Liver Disease in Sickle Cell Patients
Sickle cell patients are prone to liver injury from viral hepatitis, iron overload, hypoxic injury, common bile duct obstruction, biliary sludge, acute cholecystitis, choledocholithiasis, and the hepatic crisis syndromes. Thus it is important to be familiar with these entities as etiologies of liver disease in sickle cell patients. Viral hepatitis is prevalent in the sickle cell population. This is due to the large quantities of blood products that this population receives. Reported rates for hepatitis В infection have ranged from 15% to 20%. In the United States 1.8% of all patients with sickle cell disease are infected with the hepatitis С virus. The progression to cirrhosis in sickle cell patients with hepatitis С is not known. There have been no American studies that have examined the prevalence of hepatitis A in sickle cell patients. Sickle cell patients should be tested for the antibodies for hepatitis А, В and C. If their serologies are negative they should receive the vaccinations for hepatitis A and B.
Patients with sickle cell disease who receive blood transfusions are at risk for developing iron overload by the time they reach adulthood. Measurement of serum ferritin is the most common method used to determine body iron stores. However, in sickle cell patients, interpretation of ferritin values may be complicated by changes in body iron burden. This may occur during infection, ineffective erythropoiesis, or hemolysis. Studies have shown that the best way to determine iron stores in sickle cell patients is to keep accurate records of blood transfusions and serial determinations of ferritin levels in a steady state. Desferal chelation is the only therapy that has proven to effectively treat hemochromatosis induced by red blood cell transfusions. This results in an increase of urinary and biliary excretion of iron, promoting tissue recovery.
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Acute cholecystitis and choledocholithiasis present as right upper quadrant pain and fever in patients with sickle cell disease. Studies have estimated that 70% of patients with sickle cell disease develop gallstones. Biliary sludge is detectable on an abdominal ultrasound. It may be a precursor to gallstone development. Pigmented gallstones are the most common type of gallstones seen in sickle cell patients due to chronic hemolysis. The index of suspicion for the diagnosis of acute cholecystitis, choledocholithiasis, and common bile duct obstruction can be lowered with an abdominal ultrasound or CT scan of the abdomen. Elective cholecystectomy should be considered in the treatment of sickle cell patients with symptomatic gallstones. This procedure should not be performed in patients in a sickle cell pain crisis due to its high morbidity and mortality rates.