Patient 2
A 16-year-old boy with HbSC disease, who had undergone splenectomy
splenectomy at age 4 years after splenic sequestration, presented with
fever, sore throat, fatigue, headache, and chest pain. There was no
lymphadenopathy. WBC count was 13
x 103/µL, with
increased atypical lymphocytes (1.1 x 103/µL), Hb was
12.9 g/dL, and platelet count was 409
x 109/L.
Creatinine was elevated at 1.4 mg/dL, possibly reflecting dehydration,
but electrolytes were within normal limits. Liver function tests were
not performed at that time. Nasopharyngeal viral nucleic acid swab and
blood culture were negative, and chest x-ray showed clear lungs. He
received ceftriaxone and intravenous fluids. His pain was controlled
with oral analgesics, so he was discharged home.
He returned 2 days later with persistent fever and progressive lower
back, chest, and upper abdominal pain. His WBC had increased to 26.3
x 103/µL with a
persistent atypical lymphocytosis. Hb was 10.8 g/dL and platelet count
was 297 x 109/L. Serum chemistries were notable for
elevated bilirubin (4.5 mg/dL), ALT (366 U/L), AST (375 U/L), and γGT
(74 U/L). Creatinine was slightly lower than his previous visit at 1.04
mg/dL. Plasma lipase and amylase were normal. His chest x-ray remained
clear, and abdominal ultrasound showed a normal gallbladder. He was
admitted for intravenous analgesia and monitoring.
Over the next 12 hours, his pain intensified and his abdomen became
distended. Hepatic ultrasound demonstrated hepatomegaly with low hepatic
arterial velocity and resistive index, suggestive of sequestration. He
developed hypoxemic respiratory failure and was intubated. Chest x-ray
showed interval development of right hilar consolidation and basilar
opacities. Serial laboratory tests demonstrated a progressive
leukocytosis and marked increase in ALT and total bilirubin (Fig 1)
consistent with acute hepatitis. Plasma ammonia was normal.
He underwent an automated red cell exchange on the first hospital day
with goal Hb 10 g/dl and Hb S <30%. Shortly thereafter, he
developed hypotension requiring vasopressors. He had hypocalcemia likely
caused by inability to hepatically metabolize the citrate
anticoagulation utilized in the erythrocytapheresis procedure. He
developed laboratory evidence of coagulopathy (aPTT = 52 s, INR = 2.3)
on day 3, without clinical bleeding, that resolved over the ensuing
week. He required continuous renal replacement therapy for acute kidney
injury with creatinine > 5 mg/dL and circulatory overload.
He received additional erythrocyte transfusions to maintain his Hb
>8 g/dL.
His clinical course was consistent with intrahepatic cholestasis, a
severe form of hepatopathy marked by liver synthetic dysfunction,
coagulopathy and often multi-organ failure.1, 5 He
gradually improved and was extubated on day 9 but was persistently
febrile, so he underwent an evaluation for infection. Cytomegalovirus
IgM and IgG were negative. EBV viral capsid antigen (VCA) IgM and IgG
were positive on day 10, suggesting that acute EBV infection incited his
critical illness. Reinforcing this premise, a convalescent EBV VCA IgM
drawn 5 months later was negative. A 6-month regimen of chronic
transfusion therapy was administered to facilitate organ healing.