Patient 1
A 14-year-old girl with hemoglobin SC (HbSC) disease and a history of
two to three hospitalizations per year for vaso-occlusive episodes
presented with fever and pain in her back, chest, and legs. There was no
lymphadenopathy, pharyngitis, or hepatomegaly. White blood cell (WBC)
count was 12 x 103/µL, with numerous atypical
lymphocytes (1.2 x 103/µL), and Hb was slightly lower
than her baseline of 10-10.5 g/dL. Nasopharyngeal viral nucleic acid
swab and blood culture were negative, and chest x-ray showed no
infiltrates. Ceftriaxone and pain medications were administered, and she
was hospitalized.
Following admission, she had nausea, persistent fever, right upper
quadrant (RUQ) pain, and developed dark urine. By day 3, her liver edge
was tender and palpable 2-3 cm below the right costal margin, and
progressive anemia (Hb 8.9 g/dL) and thrombocytopenia (platelet count 67
x 109/L) were evident (Fig 1). Hepatic ultrasound
showed moderate hepatomegaly with an echogenic liver but no thrombosis.
Laboratory testing showed elevated serum levels of γ-glutamyltransferase
(γGT, 155 U/L), total bilirubin (11.8 mg/dL), conjugated bilirubin (6.8
mg/dL), alanine transaminase (ALT,
781 U/L), and aspartate transaminase (AST, 1360 U/L). Plasma fibrinogen
and activated partial thromboplastin time (aPTT) were normal. Her
prothrombin time/international normalized ratio (INR) was borderline
elevated. Laboratory testing for hepatitis A/B/C infection was negative.
Parvovirus polymerase chain reaction (PCR) and adenovirus PCR were
negative. Cytomegalovirus IgG was positive but IgM was negative.
Quantitative blood PCR assay for EBV DNA showed significant elevation at
7020 IU/mL.
The clinical picture was most consistent with hepatic sequestration
triggered by EBV. To avoid the phenomenon of reverse
sequestration,10 she received a simple transfusion of
one unit of packed red blood cells with prompt and sustained improvement
in her Hb level. Tests of liver function normalized by one week after
hospital discharge.