Abstract
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.
Keywords: Adaptive immunity; COVID-19; Inborn errors of immunity; SARS-CoV-2; Severe combined immunodeficiency.
【저자키워드】 COVID-19, SARS-CoV-2, Adaptive immunity, inborn errors of immunity, severe combined immunodeficiency, 【초록키워드】 SARS CoV-2, convalescent plasma, Adaptive immunity, Mutation, adaptive, Immunity, immunodeficiency, B cells, NK cells, RT-PCR, Antigen, Infant, Gene therapy, Nasopharyngeal swab, Hepatitis, Transplant, nasopharyngeal swabs, Patient, Allogeneic, Natural killer cells, respiratory, Donor, specific antibodies, opportunistic Infections, CD34, combined immunodeficiency, Conditioning, severe combined immunodeficiency, respiratory symptoms, transplantation, stem cell, dysfunction, deficiency, IL-2 receptor, deficiencies, SARS-CoV-2 specific antibodies, SARS CoV, respiratory symptom, disorder, SCID, Cell, X-linked, selected, resulting, caused, clinically, treated, Inborn error, infected with SARS-CoV-2, 【제목키워드】 SARS-COV-2 infection, X-linked,