Two X-linked agammaglobulinemia patients develop pneumonia as COVID-19 manifestation but recover
Open accessAbstract
Background: The recent SARS‐CoV‐2 pandemic, which has recently affected Italy since February 21, constitutes a threat to normal subjects, as the coronavirus disease‐19 (COVID‐19) can manifest with a broad spectrum of clinical phenotypes ranging from asymptomatic cases to pneumonia or even death. There is evidence that older age and several comorbidities can affect the risk to develop severe pneumonia and possibly the need of mechanic ventilation in subjects infected with SARS‐CoV‐2. Therefore, we evaluated the outcome of SARS‐CoV‐2 infection in patients with inborn errors of immunity (IEI) such as X‐linked agammaglobulinemia (XLA). Methods: When the SARS‐CoV‐2 epidemic has reached Italy, we have activated a surveillance protocol of patients with IEI, to perform SARS‐CoV‐2 search by nasopharyngeal swab in patients presenting with symptoms that could be a manifestation of COVID‐19, such as fever, cough, diarrhea, or vomiting. Results: We describe two patients with X‐linked agammaglobulinemia (XLA) aged 34 and 26 years with complete absence of B cells from peripheral blood who developed COVID‐19, as diagnosed by SARS‐CoV‐2 detection by nasopharyngeal swab, while receiving immunoglobulin infusions. Both patients developed interstitial pneumonia characterized by fever, cough, and anorexia and associated with elevation of CRP and ferritin, but have never required oxygen ventilation or intensive care. Conclusion: Our report suggests that XLA patients might present with high risk to develop pneumonia after SARS‐CoV‐2 infection, but can recover from infection, suggesting that B‐cell response might be important, but is not strictly required to overcome the disease. However, there is a need for larger observational studies to extend these conclusions to other patients with similar genetic immune defects.
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Individuals with agammaglobulinemia or pharmaceutical depletion of B cells generally experience an uncomplicated COVID-19 disease course.
“Furthermore, individuals with agammaglobulinemia or pharmaceutical depletion of B cells generally experience an uncomplicated COVID-19 disease course.33,37,38”
From Impact of SARS-CoV-2 variants on the total CD4+ and CD8+ T cell reactivity in infected or vaccinated individuals (2021) · cites this paper as [37]
Some individuals with inherited or treatment-related B cell deficiencies who did not produce neutralizing antibodies still recovered from COVID-19.
“patients who failed to develop neutralizing antibodies, in some cases because of inherited or treatment-induced B cell deficiencies, have recovered from COVID-19 (Soresina et al., 2020; Wu et al., 2020; Wurm et al., 2020).”
From Rapid induction of antigen-specific CD4+ T cells is associated with coordinated humoral and cellular immunity to SARS-CoV-2 mRNA vaccination (2021) · cites this paper as Soresina et al., 2020
Two unrelated adults in Italy with agammaglobulinemia and no circulating B cells developed COVID-19 and fully recovered.
“Two unrelated adults in Italy with agammaglobulinemia and no circulating B cells developed COVID-19 and fully recovered from infection (Soresina et al., 2020), suggesting that antibodies can be dispensable for protective immunity to SARS-CoV-2 in otherwise healthy adults.”
From Adaptive immunity to SARS-CoV-2 and COVID-19 (2021) · cites this paper as Soresina et al., 2020
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- Authors
- Soresina, A., Moratto, D., Chiarini, M., Paolillo, C., Baresi, G., Focà, E., Bezzi, M., Baronio, B., Giacomelli, M., & Badolato, R.
- Affiliations
- Pediatric Immunology Unit Brescia Italy
- Flow Cytometry Clinical Chemistry Laboratory Brescia Italy
- Azienda Socio Sanitaria Territoriale degli Spedali Civili di Brescia (IT)
- University of Brescia (IT)
- Istituto Clinico Sant'Ambrogio (IT)
- Corresponding author
- Badolato, Raffaele
- Venue
- Pediatric Allergy and Immunology
- Publisher
- Wiley
- Published
- 2020-05-19
- Volume
- 31
- Issue
- 5
- Pages
- 565-569
- ISSN
- 0905-6157 (ISSN-L)
- 1399-3038
- PMC
- PMC7264678
- OpenAlex
- W3016322638
- Cited by
- 327
- References
- 12
- Citations by year
- 2026: 3 · 2025: 13 · 2024: 22 · 2023: 39 · 2022: 77 · 2021: 99 · 2020: 74
- FWCI
- 9.47
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Pediatric Allergy and Immunology (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Institutional Research Information System (Università degli Studi di Brescia) (submittedVersion, public-domain)
- Topics
- Health Sciences › Medicine › Infectious Diseases › COVID-19 Clinical Research Studies
- Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
- Health Sciences › Medicine › Infectious Diseases › SARS-CoV-2 and COVID-19 Research
- Keywords
- X-linked agammaglobulinemia, COVID-19 pandemic, SARS-CoV-2, interstitial lung disease, primary immunodeficiency, B cell deficiency, immunoglobulin replacement therapy, disease susceptibility, recovery from infection
- MeSH terms
- Betacoronavirus, COVID-19, SARS-CoV-2, Adult, Agammaglobulinemia, Anti-Bacterial Agents, Enzyme Inhibitors, Humans, Hydroxychloroquine, Immunization, Passive, Italy, Male, Pneumonia, Viral, Treatment Outcome, Coronavirus Infections, Genetic Diseases, X-Linked, Pandemics
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