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.