Jaundice Accompanied by Giant Cell Hepatitis and Eosinophilia in Childhood

CASE HISTORY

A 9-year-old white girl was referred for evaluation and management of persistent jaundice, elevated transaminases, and peripheral eosinophilia. Her jaundice was associated with pale stools and dark urine, which were noted for the first time 10 weeks earlier. At that time, there was no accompanying fever, diarrhea, or rash. No history of infectious contact, travel, or drug ingestion could be elicited. The jaundice had persisted since presentation, although it had reduced in intensity. She continued to remain asymptomatic and was on no medication. The only past medical history of note was a streptococcal throat infection. Pharyngitis occurred 4 months before the onset of jaundice and had been a prolonged illness requiring antibiotics, including amoxicillin for approximately 6 weeks. Otherwise, her past medical history was not significant. There was no relevant family history. She was not known to have any allergies.

On examination, she appeared mildly jaundiced but was otherwise well with no dysmorphic features. Her weight was just below the 50th percentile, and her height was just above the 25th percentile for her age. Examination of ear, nose, and throat revealed enlarged tonsils with no congestion. No significant lymphadenopathy or cutaneous stigmata of liver disease were noted. Her chest was clear, with no murmurs. The abdomen was soft. The liver was palpable 2 cm in the midline, and the spleen was palpable 2 cm below the costal margin. There were no ascites, and she was neurologically intact.

At presentation, blood tests revealed hemoglobin of 11.4 g/dL; white blood cell count of 7.9 × 10³/mL, with 27% eosinophils; platelet count of 293 × 10³ /mL; total bilirubin 7.1 mg/dL (0.1 to 0.2 mg/dL); aspartate aminotransferase (AST) 691 U/L (5 to 42 U/L); alanine aminotransferase (ALT) 473 U/L (5 to 45 U/L); cholesterol 259 mg/dL (100 to 200 mg/dL); triglycerides 301 mg/dL (10 to 140 mg/dL); hepatitis A virus immunoglobulin M (IgM) nonreactive; hepatitis B core antibody reactive; hepatitis B surface antigen nonreactive; hepatitis B core antibody nonreactive; hepatitis C virus enzyme-linked immunosorbent assay nonreactive; and herpes simplex virus-1 IgG negative. Toxocara serologies were negative, as were stool ova and parasites. Tissue transglutaminase was negative, ceruloplasmin was 63 mg/dL (22 to 58 mg/dL), IgG was 960 mg/dL (589 to 1717 mg/dL), IgA was 226 mg/dL (41 to 252 mg/dL), IgM was 277 mg/dL (49 to 270 mg/dL), serum IgE was 12 IU/mL (1 to 365 IU/mL), smooth muscle antibody (SMA) was positive 1:20, antinuclear antibody (ANA) was positive 1:40 homogenous patterns, liver kidney microsomal antibody (anti-LKM) negative, α₁-antitrypsin (AAT) phenotype was MS. An abdominal ultrasound had showed a normal liver and gallbladder, prominent spleen, and normal kidneys. A percutaneous liver biopsy had been performed, and the histology was consistent with, but not classic for, autoimmune hepatitis. There was chronic inflammatory cell infiltrate with rare plasma cells, lobular inflammation, and piecemeal necrosis. The hepatocytes, however, were noted to have abundant cytoplasm and multinucleation.

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