Authors: Randolph P. Matthews, MD, PhD; Barbara A. Haber, MD; Petar Mamula, MDSeries Editors: David A. Piccoli, MD; Petar Mamula, MD

Disclosures

May 03, 2004

Case Presentation

A 5-year-old girl, previously healthy, presented to her primary pediatrician with a 1-week history of intermittent fever to 38.9°C, fatigue, cough, congestion, and a diffuse skin rash. In addition to a whole-body macular rash, massive hepatosplenomegaly was noted on physical exam. She was referred to a gastroenterologist for further evaluation.

There was no history of jaundice, pruritus, easy bruising or bleeding, fractures, weight loss, or abdominal pain. Past medical history was normal and the patient had been growing and developing well. There was no family history of liver disease or cancer. The patient was the oldest of 3 sisters who were all healthy. There was no history of allergies to any medication, and the patient was not taking any medications or over-the-counter preparations. Her parents stated that abdominal protuberance had been present since infancy.

Vital signs were normal and she was afebrile. The patient appeared healthy, well developed, and well nourished. There was no scleral icterus or jaundice noted. Several small, < 2-cm mobile nontender lymph nodes were palpable in the anterior cervical area. A macular rash was noted on the trunk. Chest and heart exam were normal. The abdomen was nontender but distended, with the liver extending 15 cm below the right costal margin with a span of 20 cm, and the spleen extending 8 cm below the left costal margin.

Laboratory studies were obtained and revealed a normal complete blood count with differential analysis, except for mild normochromic, normocytic anemia with a hemoglobin level of 9.9 g/dL. Erythrocyte sedimentation rate was elevated at 36 mm/hr and the blood chemistry panel was normal. Liver function tests revealed normal bilirubin, albumin, alkaline phosphatase, international normalized ratio, and gamma glutamyltransferase, while serum alanine aminotransferase and aspartate aminotransferase levels were increased at 229 U/L and 133 U/L, respectively. Her cholesterol level was high, at 219 mg/dL, as was her triglyceride level, at 163 mg/dL. Additional laboratory studies demonstrated negative hepatitis A, B, and C serologies, and negative Epstein-Barr virus, toxoplasmosis, syphilis, and autoimmune hepatitis serologies. Alpha-1 antitrypsin phenotype and ceruloplasmin level were normal and cytomegalovirus antigenemia was negative. The Lyme IgM antibody was positive by enzyme immunoassay and Western blot analysis. Abdominal ultrasound and computed tomography scan demonstrated massive hepatosplenomegaly but no focal abnormalities or lymphadenopathy.

The patient was treated with amoxicillin for suspected Lyme disease. Prior to the initiation of this treatment, her rash and fever had resolved spontaneously. Three weeks later her liver and spleen size were unchanged. In the interim, she had had a normal bone marrow aspirate and biopsy. At this point, a liver biopsy was performed (Figure 1).

Results of liver biopsy; histology. Light microscopy, hematoxylin, and eosin staining. (Courtesy of Dr. P. Russo, Department of Pathology, The Children's Hospital of Philadelphia.)

How do you interpret the findings of the liver biopsy?

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