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Case Reports
. 2021 Mar:224:108662.
doi: 10.1016/j.clim.2020.108662. Epub 2021 Jan 4.

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

Nicolai S C van Oers  1 , Natasha W Hanners  2 , Paul K Sue  3 , Victor Aquino  3 , Quan-Zhen Li  4 , John W Schoggins  5 , Christian A Wysocki  6
Affiliations
Case Reports

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

Nicolai S C van Oers et al. Clin Immunol. 2021 Mar.

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.

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Conflict of interest statement

None.

Figures

Fig. 1
Fig. 1
A. IL2RG gene map with patient's mutation at the intron 5 splice acceptor. B. Time course of events throughout the patient's life. Grey filled horizontal bar represents the hospitalization. Small yellow triangles represent positive RT-PCR tests for SARS-CoV-2. Small blue triangles indicate negative tests. C. Heatmap of IgG binding activity in patient serum at various timepoints after convalescent plasma, in samples from non-SARS-CoV-2 infected infants, and COVID-19 patients from a patient registry. D. Relative IgG binding activity for SARS-CoV-2 spike proteins S1 and S2, RBD, NCP as well as S2 extracellular domain (ECD), 3CL and Papain-like proteases (Plpro), and M and Envelope proteins. Y axes represent net signal intensity (NSI) in which background staining is subtracted from each sample. (For interpretation of the references to colour in this figure legend, the reader is referred to the web version of this article.)
See this image and copyright information in PMC

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