Case 1
A previously healthy ex-full-term two-year-old female presented with a
two-day history of abdominal pain, non-bloody, non-bilious emesis, and
jaundice. Four months prior to presentation, both the patient and her
unvaccinated parents tested positive for SARS-CoV-2 via RT-PCR nasal
swab. They all had mild cough and runny nose that resolved over a ten
day period. Three days prior to her presentation, she developed cough,
congestion, jaundice and tactile fever.
The patient’s medical history was significant for minor viral upper
respiratory infections. She had never been hospitalized nor had surgery,
did not take daily medications or endorse any allergies. The family
history was negative for liver diseases.
The patient’s vital signs on initial presentation were within
appropriate limits for age. She had scleral icterus, generalized
jaundiced skin, and multiple bilateral lower extremity bruises each
measuring 2 to 3 cm in diameter and concentrated at the mid-shins. Her
lung examination was normal. She had no hepatomegaly nor splenomegaly,
and the rest of her physical exam was otherwise unremarkable.
The patient’s initial diagnostic assessment was notable for
transaminitis with ALT: 1,902 U/L, AST: 2,158 U/L, leukopenia 4.9 x
103/uL, thrombocytopenia: 75 x
103/uL, and a prolonged INR at 1.4. Her respiratory
viral panel was positive for SARS-CoV-2 and Parainfluenza 3.
Her abdominal ultrasound demonstrated an echogenic liver parenchyma with
no focal lesions.
Liver biopsy showed acute hepatitis with patchy hepatic necrosis and
dropout. Her complete blood count stayed stable but her transaminitis
rose alongside INR. INR peaked at 5.4 on the ninth day of admission
despite Vitamin K infusions. Ammonia level went up to 250 ug/dL and she
started manifesting hepatic encephalopathy. A second liver biopsy was
performed on day 10 showing massive necrosis. Immunostains revealed a
predominant population of CD8+ T-cells that were present in both the
lobules and portal tracts. There were fewer CD4+ T-cells and CD20+
B-cells (which were primarily located within the portal tracts). In
addition, the CD163 immunostain highlighted a significant infiltrate of
macrophages within the lobules. She was urgently listed for a liver
transplant as status 1A.
On the 12th day of admission, the patient received an
orthotopic left lateral liver transplant.
The patient tolerated surgery well and made significant progress in her
post-transplant course. However, her anemia and thrombocytopenia
worsened to hemoglobin < 5.6 g/dL and platelets < 10
x103/uL requiring serial transfusions of packed red
blood cells and platelets. She also developed leukopenia and neutropenia
with absolute neutrophil count (ANC) below 500. A bone marrow biopsy
confirmed the diagnosis of idiopathic aplastic anemia.
She was treated per aplastic anemia protocol with intravenous
immunoglobulin (IVIG), anti-thymocyte globulin (ATG),
methylprednisolone. Her immunosuppression regimen was switched from
tacrolimus to cyclosporine.
At three months post-treatment for aplastic anemia, her hemoglobin
stabilized in the 9-11g/dL range, platelets ranged between 100 -120 x
103/uL, and her liver chemistry panel remained within
normal ranges.