Background:

The pathogenesis of infection triggered by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has been intensively investigated, constituting a challenge on a global scale. It is known that the effectiveness and magnitude of both innate and acquired immune responses to SARS-CoV-2 infection determine the severity of the clinical presentation. Literature on the evolution of SARS-CoV-2 infection in patients with X-linked agammaglobulinemia is still scarce.

Objective:

To report the spectrum of clinical manifestations associated with SARS-CoV-2 infection in patients with X-linked followed in a tertiary hospital.

Methods:

This is an observational retrospective study of patients with X-linked agammaglobulinemia with SARS-CoV-2 infection diagnosed between March 2020 and July 2022.

Results:

Among 13 patients under follow-up, nine

patients were included, with a mean age of 32 ± 6.1 years. Two patients reported a second SARS-CoV-2 infection during the study. All patients were on polyvalent immunoglobulin G replacement therapy. Seven patients presented risk factors for severe COVID-19 disease: bronchiectasis (n=5), insulin-dependent diabetes (n=1), active smoking (n=1), obesity (n=1), cerebrovascular disease (n=1), immunosuppressive therapy (n=1). COVID-19 infection was mild in most patients (asymptomatic infection n=1, mild n=9, moderate n=1). The most frequently affected systems were the upper (n=6) and lower (n=5) respiratory airways, with fever (n=7) and nasal congestion (n=6) being the most frequent manifestations. Bacterial infection was reported in 4 patients. One patient underwent therapy with convalescent plasma, with tolerance and a favorable clinical course.

Conclusion:

In this series of patients with X-linked agammaglobulinemia, a high prevalence of SARS-CoV-2 infection is reported, highlighting a mild severity outcome in almost all patients.

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