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

• Published in: Clinical immunology (Orlando, Fla.) Vol. 224; p. 108662
• Main Authors: van Oers, Nicolai S.C., Hanners, Natasha W., Sue, Paul K., Aquino, Victor, Li, Quan-Zhen, Schoggins, John W., Wysocki, Christian A.
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-03-2021
• Subjects: Adaptive immunity; COVID-19; COVID-19 - complications; COVID-19 - therapy; COVID-19 Serotherapy; Hepatitis - virology; Humans; Immunization, Passive - methods; Inborn errors of immunity; Infant; Male; SARS-CoV-2; Severe combined immunodeficiency; Severe Combined Immunodeficiency - complications; COVID-19; SARS-CoV-2; Adaptive immunity; Severe combined immunodeficiency; Inborn errors of immunity
• ISSN: 1521-6616; 1521-7035
• DOI: 10.1016/j.clim.2020.108662

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.