Patient with Good’s syndrome and COVID-19. Report of a clinical case
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Keywords
SARS-CoV-2 , Coronavirus Infections, Thymoma, Severe Combined Immunodeficiency
Abstract
Background: The consequences of SARS-CoV-2 infection in patients with primary (now called “inborn errors of immunity”) or secondary immunodeficiencies is still a matter of debate. There are few reports in the literature of patients with Good’s syndrome and SARS-CoV-2 infection with variable outcomes.
Clinical case: A 51-year-old male with diagnosis of Good’s syndrome treated with intravenous human immunoglobulin (IVIG) at a replacement dose with application every 21 days and prophylaxis for P. jirovecii with trimethoprim/ sulfamethoxazole due to profound lymphopenia at expense of T CD4+ lymphocytes who presented initially mild disease (RT-PCR+) that progressed to pneumonia with acute respiratory failure and required advanced airway management and admission to the ICU with a fatal outcome due to superinfection after 14 days hospitalized.
Conclusion: It has been documented in patients with humoral immunodeficiencies a better prognosis for developing less intense cytokine release syndrome. The alteration in cellular immunity, especially lymphopenia at the expense of CD4+ T lymphocytes, may be associated with a worse prognosis as the response against viruses is compromised as well as high susceptibility to superinfection by opportunistic agents such as P. aeruginosa and Mucor sp. For this reason, we must maintain close surveillance in patients with inborn errors of immunity with cellular defects, as is the case of patients with Good’s syndrome who present with COVID-19.
References
Shields AM, Burns SO, Savic S, Richter AG; UK PIN COVID-19 Consortium. COVID-19 in patients with primary and secondary immunodeficiency: The United Kingdom experience. J Allergy Clin Immunol. 2021;147(3):870-75. DOI: 10.1016/j. jaci.2020.12.620.
Kelesidis T, Yang O. Good’s syndrome remains a mystery after 55 years: a systematic review of the scientific evidence. Clin Immunol. 2010;135(3):347-63. DOI: 10.1016/j.clim.2010.01.006.
Bousfiha A, Jeddane L, Picard C, Al-Herz W, Ailal F, Chatila T, et al. Human inborn errors of immunity: 2019 update of the IUIS phenotypical classification. J Clin Immunol. 2020;40(1):66-81. DOI: 10.1007/s10875-020-00758-x.
Malphettes M, Gérard L, Galicier L, Bouthoul D, Asli B, Szalat R, et al. Good syndrome: an adult-onset immunodeficiency remarkable for its high incidence of invasive infections and autoimmune complications. Clin Infect Dis. 2015;61(2): e13- e19. DOI: 10.1093/cid/civ269.
Ryman NG, Burrow L, Bowen C, Carrington C, Dawson A, Harrison NK. Good›s syndrome with primary intrapulmonary thymoma. J R Soc Med. 2005;98(3):119-20. DOI: 10.1258/ jrsm.98.3.119.
Pozzi M, Baronio M, Bianchi Janetti MB, Gazzurelli L, Moratto D, Chiarini M, et al. Fatal SARS-CoV-2 infection in a male patient with Good›s syndrome. Clin Immunol. 2021;223:108644. DOI: 10.1016/j.clim.2020.108644.
London J, Boutboul D, Lacombe K, Pirenne F, Heym B, Zeller V, et al. Severe COVID-19 in patients with B cell alymphocytosis and response to convalescent plasma therapy. J Clin Immunol. 2021;41(2): 356-61. DOI: 10.1007/ s10875-020-00904-5.
Lindahl H, Smith CIE, Bergman P. COVID-19 in a patient with Good›s syndrome and in 13 patients with common variable immunodeficiency. Clinical Immunology Communications. 2021;1(2021):20-24. DOI: 10.1016/j.clicom.2021.08.003.
Valásquez-Ortiz MG, O›Farrill-Romanillos PM, Berrón-Ruiz L. Conceptos generales de las inmunodeficiencias humorales. Rev Alerg Mex. 2020;67(2):142-64. DOI: 10.29262/ram. v67i2.763.
Jansen A, van Deuren M, Miller J, Litzman J, De Gracia J, Sáenz-Cuesta M, et al. Prognosis of Good syndrome: mortality and morbidity of thymoma associated immunodeficiency in perspective. Clin Immunol. 2016;171(2016):12-17. DOI: 10.1016/j.clim.2016.07.025.
Pu C, Sukhal S, Fakhran S. Humoral immunity in bronchiectasis: finding Good’s syndrome. Case Rep Pulmonol. 2015; 2015:531731. DOI: 10.1155/2015/531731.
Kelleher P, Misbah SA. What is Good›s syndrome? immunological abnormalities in patients with thymoma. J Clin Pathol. 2003; 56(1): 12-16. DOI: 10.1136/jcp.56.1.12.
Lai Y, Tan T. Atypical presentation of Good syndrome: acute hepatitis from hepatitis B virus reactivation. Asia Pac Allergy. 2020;10(4):e37. DOI: 10.5415/apallergy.2020.10.e37.
Duarte M, Faria L, Patronillo C, da Costa-Fernandes S, Seara V. A case of severe COVID–19 in a patient with Good`s syndrome. Eur J Case Rep Intern Med. 2021;8(10):002976. DOI: 10.12890/2021_002976.
Ponsford MJ, Shillitoe BMJ, Humphreys IR, Gennery A, Jolles S. COVID–19 and X–linked agammaglobulinemia(XLA)–insights from a monogenic antibody deficiency. Cuur Opin Allergy Clin Immunol 2021;21(6):525-34. DOI: 10.1097/ ACI.0000000000000792.
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