4.7 Article

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

Journal

CLINICAL IMMUNOLOGY
Volume 224, Issue -, Pages -

Publisher

ACADEMIC PRESS INC ELSEVIER SCIENCE
DOI: 10.1016/j.clim.2020.108662

Keywords

SARS-CoV-2; COVID-19; Severe combined immunodeficiency; Inborn errors of immunity; Adaptive immunity

Categories

Funding

  1. National Institutes of Health [RO1 Al114523]
  2. NIH [K08 AI132751-01A1]
  3. Department of Immunology at UT Southwestern Medical Center
  4. Jeffrey Modell Foundation

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X-linked severe combined immunodeficiency (X-SCID) is a severe immune disorder caused by genetic mutations, with patients facing opportunistic infections. An infant with X-SCID contracted SARS-CoV-2 before receiving a stem cell transplant, but ultimately recovered after treatment.
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.

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