SARS-CoV-2 infection associated with hepatitis in an infant with X-linked severe combined immunodeficiency

X-linked severe combined immunodeficiency (X-SCID) is a disorder of adaptive immunity caused by mutations in the IL-2 receptor common gamma chain gene resulting in deficiencies of T and natural killer cells, coupled with severe dysfunction in B cells. X-SCID is lethal without allogeneic stem cell transplant or gene therapy due to opportunistic infections. An infant with X-SCID became infected with SARS-CoV-2 while awaiting transplant. The patient developed severe hepatitis without the respiratory symptoms typical of COVID-19. He was treated with convalescent plasma, and thereafter was confirmed to have SARS-CoV-2 specific antibodies, as detected with a microfluidic antigen array. After resolution of the hepatitis, he received a haploidentical CD34 selected stem cell transplant, without conditioning, from his father who had recovered from COVID-19. SARS CoV-2 was detected via RT-PCR on nasopharyngeal swabs until 61 days post transplantation. He successfully engrafted donor T and NK cells, and continues to do well clinically. •The presence of uncorrected X-linked SCID in an infant who became infected with SARS-CoV-2 lead to a mild COVID-19 disease course.•COVID-19 disease in this X-SCID infant was characterized by hepatitis, and prolonged viral shedding.•The prolonged period of viral shedding continued post-transplant, with viral clearance correlating with donor T and NK cell engraftment.