Acute Hepatic Sequestration in Sickle Cell Disease
A 29-year-old black male presented to the emergency room with a complaint of back pain, chest pain, abdominal pain, and dysuria for three days. He had associated fever, chills, and dark urine. On presentation, his temperature was 40°C, the pulse was 120 beats per minute, and the respiratory rate was 32 breaths per minute. The blood pressure was 114/52, and his oxygen saturation was 89% while breathing room air. His physical examination revealed jaundiced skin, scleral icterus, tachycardia, right upper quadrant tenderness on palpation, a liver span 16 cm to percussion, and costovertebral angle tenderness bilaterally. His past medical history included sickle cell disease, tuberculosis with completed treatment in 2000, and a positive hepa-titis-B core antibody in March of2001. His social history included smoking one pack of cigarettes daily and occasional marijuana use, but he denied any intravenous drug or alcohol use. His blood chemistry values were total bilirubin of 49.8 mg/dl, direct bilirubin 28.5, indirect bilirubin 21.3, and an ammonia level of 81 (imol/L. The levels of urea nitrogen, creatinine, glucose, and electrolytes were normal. The enzymes showed an alkaline phosphatase (ImU/ml) 276, lactate dehydrogenase (U/liter) 477, gamma-glutamyl transferase (U/liter) 524, aspartate aminotransferase (U/liter) 58, alanine aminotransferase (U/liter) 61. His coagulation studies were partial-thromboplastin time (PTT) 38.1, and an International Normalized Ratio (INR) of 1.18. His hematocrit was 17%, and he had a serum white blood cell count of 24,000 with 14% bands. His urinalysis showed bilirubin, positive nitrate, positive leukocyte esterase, and numerous red and white blood cells. His urine culture, hepatitic-C virus antibody, and urine drug screen were negative. He had positive blood cultures for Escherichia coli. An abdominal ultrasound showed hepatomegaly and minimal gallbladder sludge, but normal intra and extrahepatic ducts. A subsequent computed tomographic (CT) exam of the abdomen, performed without the use of contrast material, showed hepatomegaly at 20 cm without lesions, absent spleen, and no biliary dilatation.
The patient was admitted to the inpatient ward. Treatment began with intravenous normal saline and parenteral levafloxacin for his bacteremia. He received a six-unit, red blood cell partial exchange transfusion and a transfusion of three units of packed red blood cells. Lactulose was given for his elevated ammonia. After treatment, his hematocrit increased to 29%, his white blood cell count decreased to 12,000, and he remained afebrile after hospital day six. His total bilirubin decreased to 4.9 mg/dl, with a direct fraction of 2.5. The transaminases normalized after hospital day five. The patient left the hospital against medical advice (AMA) after hospital day 17, prior to receiving a liver biopsy.
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Epidemiology and Clinical Features
Homozygous sickle cell anemia affects 1 in 600 African-American babies, and sickle cell trait affects 8-10% of African Americans. Liver disease is apparently prevalent in sickle cell disease. It has been estimated that hepatic complications affect 10% of patients with sickle cell disease who are admitted to the hospital. In an autopsy study on 70 patients with sickle cell anemia, sickle thalassemia, and sickle С disease, hepatomegaly was found in 91% of the patients.
Cases have shown that it is possible that infections, fever, and pain may play a role as precipitating factors in sickle cell patients that present with an intrahepatic vaso-occlusive syndrome. The right upper quadrant hepatic syndrome patient typically complains of right upper quadrant pain and fever. The clinical exam is significant for jaundice and hepatic enlargement. Serum bilirubin levels are usually less than 15 mg/dl. The plasma alanine aminotransferase and aspartate aminotransferase levels are elevated.
Sixteen cases of intrahepatic cholestasis in sickle cell disease have been reported. This condition is potentially catastrophic. The clinical features include fever, right upper quadrant pain, encephalopathy, and jaundice. The laboratory values show leukocytosis, elevated lactate dehydrogenase, renal failure, elevated transaminases and prolongation of the prothrombin, and partial thromboplastin time.
Acute hepatic sequestration is a rarely recognized complication of vaso-occlusive crisis. It is characterized by a presentation of right upper quadrant pain. A smooth, tender, enlarged liver is noted on physical exam. The lab values show a decrease in the hemoglobin/hematocrit. An increase in reticulocyte count is also seen. viagra soft