Introduction: X-linked agammaglobulinemia (XLA) presents challenges in relation to SARS-CoV-2 infection: prolonged virus persistence, limited antibody response to vaccination and possible auto-inflammation. We describe the case of a 17-year-old Brazilian boy with XLA with SARS-CoV-2 infection at 16 months and evolution to an autoinflammatory disease, despite vaccination and a robust cellular immune response.
Presentation of the case: SARS-CoV-2 infection was confirmed on December 20, 2020 by RT-PCR. The patient was admitted to the hospital on January 24, 2021, with a 4-day history of fever. He had several complications and developed significant respiratory discomfort, requiring a few ICU stays and continuous O2 support. After 77 days, RT-PCR was negative, but fever and cough persisted. The patient was discharged from the hospital after 113 days, requiring home O2 for sleep and exercise and lost 9kg. In May 2022, he restarted with daily fever with a new positive RT-PCR and in September 2022 a positive rapid antigen test. Serum amyloid-protein-A was requested, with a high result of 518mg/L (RV 6.4mg/L). He was immunized for Covid-19 on December 8, 2021, 205 days after discharge, with mRNA-vaccines (Pfizer-BioNTech-BNT162b2) and was sampled 1 and 3-months after 3rd-dose, 1 and 4-months after 4th-dose and 1-month after 5th-dose of bivalent Pfizer. T-cell responses by ELISpot and humoral response (RBD-Wuhan neutralizing antibodies) by ELISA were evaluated. 24% neutralization was observed 1-month after 3rd-dose and 30% 3-months after 3rd-dose, which represents a negative humoral response. Despite this, T-cell responses were positive at all samples.
Discussion: We report the case of the longest duration of SARS-CoV-2 persistence in an immunocompromised patient. Maybe SARS-CoV-2 activated inflammasome pathways mimicking an autoinflammatory disease with secondary amyloidosis. The patient responded to a 3-dose immunization of SARS-CoV-2 and prior SARS-CoV-2 infection with a robust T-cell response.