Prolonged Disease Course of COVID-19 in a Patient with CTLA-4 Haploinsufficiency
Patients with primary immunodeficiencies are especially vulnerable to developing severe coronavirus disease 2019 (COVID-19) after infection with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Cytotoxic T lymphocyte antigen-4 (CTLA-4) is an important regulator of immune responses,...
| Main Authors: | , , , , |
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| Format: | Article |
| Language: | English |
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Hindawi Limited
2023-01-01
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| Series: | Case Reports in Immunology |
| Online Access: | http://dx.doi.org/10.1155/2023/3977739 |
| _version_ | 1797816613387370496 |
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| author | T. W. Hoffman H. L. Leavis B. M. Smits L. T. van der Veken D. A. van Kessel |
| author_facet | T. W. Hoffman H. L. Leavis B. M. Smits L. T. van der Veken D. A. van Kessel |
| author_sort | T. W. Hoffman |
| collection | DOAJ |
| description | Patients with primary immunodeficiencies are especially vulnerable to developing severe coronavirus disease 2019 (COVID-19) after infection with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Cytotoxic T lymphocyte antigen-4 (CTLA-4) is an important regulator of immune responses, and patients who suffer from CTLA4 haploinsufficiency have hyperactivation of effector T cells and infiltration of various organs. Overexpression of CTLA4 has been associated with a more severe disease course in patients with COVID-19, but there have only been a few reports on the disease course of COVID-19 in patients with CTLA4 haploinsufficiency. We report on a 33-year-old female with a history of immune thrombocytopenia, autoimmune haemolytic anaemia, granulomatous-lymphocytic interstitial lung disease, and common variable immunodeficiency who developed COVID-19. She was admitted and discharged from the hospital several times in the months thereafter and remained symptomatic and had a positive SARS-CoV-2 PCR for up to 137 days after the first symptoms. No SARS-CoV-2 antibodies were identified in the patients’ serum. The disease was finally controlled after repeated infusions of convalescent plasma and treatment of concurrent bacterial and fungal infections. Genetic analysis revealed a likely pathogenic variant in CTLA4, and CTLA4 expression on regulatory T-cells was low. This case illustrates that patients with primary immunodeficiencies who have a protracted disease course of COVID-19 could benefit from convalescent plasma therapy. |
| first_indexed | 2024-03-13T08:40:15Z |
| format | Article |
| id | doaj.art-9d2874d73b0a493fbea8edec918081ab |
| institution | Directory Open Access Journal |
| issn | 2090-6617 |
| language | English |
| last_indexed | 2024-03-13T08:40:15Z |
| publishDate | 2023-01-01 |
| publisher | Hindawi Limited |
| record_format | Article |
| series | Case Reports in Immunology |
| spelling | doaj.art-9d2874d73b0a493fbea8edec918081ab2023-05-30T12:28:39ZengHindawi LimitedCase Reports in Immunology2090-66172023-01-01202310.1155/2023/3977739Prolonged Disease Course of COVID-19 in a Patient with CTLA-4 HaploinsufficiencyT. W. Hoffman0H. L. Leavis1B. M. Smits2L. T. van der Veken3D. A. van Kessel4Department of PulmonologyDepartment of Rheumatology and Clinical ImmunologyDepartment of Rheumatology and Clinical ImmunologyDepartment of GeneticsDepartment of PulmonologyPatients with primary immunodeficiencies are especially vulnerable to developing severe coronavirus disease 2019 (COVID-19) after infection with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). Cytotoxic T lymphocyte antigen-4 (CTLA-4) is an important regulator of immune responses, and patients who suffer from CTLA4 haploinsufficiency have hyperactivation of effector T cells and infiltration of various organs. Overexpression of CTLA4 has been associated with a more severe disease course in patients with COVID-19, but there have only been a few reports on the disease course of COVID-19 in patients with CTLA4 haploinsufficiency. We report on a 33-year-old female with a history of immune thrombocytopenia, autoimmune haemolytic anaemia, granulomatous-lymphocytic interstitial lung disease, and common variable immunodeficiency who developed COVID-19. She was admitted and discharged from the hospital several times in the months thereafter and remained symptomatic and had a positive SARS-CoV-2 PCR for up to 137 days after the first symptoms. No SARS-CoV-2 antibodies were identified in the patients’ serum. The disease was finally controlled after repeated infusions of convalescent plasma and treatment of concurrent bacterial and fungal infections. Genetic analysis revealed a likely pathogenic variant in CTLA4, and CTLA4 expression on regulatory T-cells was low. This case illustrates that patients with primary immunodeficiencies who have a protracted disease course of COVID-19 could benefit from convalescent plasma therapy.http://dx.doi.org/10.1155/2023/3977739 |
| spellingShingle | T. W. Hoffman H. L. Leavis B. M. Smits L. T. van der Veken D. A. van Kessel Prolonged Disease Course of COVID-19 in a Patient with CTLA-4 Haploinsufficiency Case Reports in Immunology |
| title | Prolonged Disease Course of COVID-19 in a Patient with CTLA-4 Haploinsufficiency |
| title_full | Prolonged Disease Course of COVID-19 in a Patient with CTLA-4 Haploinsufficiency |
| title_fullStr | Prolonged Disease Course of COVID-19 in a Patient with CTLA-4 Haploinsufficiency |
| title_full_unstemmed | Prolonged Disease Course of COVID-19 in a Patient with CTLA-4 Haploinsufficiency |
| title_short | Prolonged Disease Course of COVID-19 in a Patient with CTLA-4 Haploinsufficiency |
| title_sort | prolonged disease course of covid 19 in a patient with ctla 4 haploinsufficiency |
| url | http://dx.doi.org/10.1155/2023/3977739 |
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