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1.
Pediatr Infect Dis J ; 40(12): e472-e474, 2021 12 01.
Article in English | MEDLINE | ID: covidwho-1447657

ABSTRACT

We present a case of a 17-year-old boy with X-linked agammaglobulinemia who had mild disease when initially infected with SARS-CoV-2 but after recovering from acute infection developed fevers and a raised erythrocyte sedimentation rate that persisted for several weeks without any ongoing respiratory symptoms. Multiple nasopharyngeal swabs were found to be negative for SARS-CoV-2 during the febrile period, but typical changes of COVID-19 on high resolution CT chest scan led to the detection of SARS-CoV-2 on RT-PCR in a sample from a bronchoalveolar lavage. His fevers completely resolved after a 5-day course of remdesivir.


Subject(s)
Agammaglobulinemia/complications , COVID-19/complications , Genetic Diseases, X-Linked/complications , Pneumonia, Viral/virology , SARS-CoV-2 , Adenosine Monophosphate/analogs & derivatives , Adenosine Monophosphate/therapeutic use , Adolescent , Alanine/analogs & derivatives , Alanine/therapeutic use , Antibodies, Viral/blood , Antiviral Agents/therapeutic use , Biomarkers/blood , Bronchoalveolar Lavage Fluid/virology , COVID-19/drug therapy , Fever , Humans , Inflammation/blood , Inflammation/metabolism , Male , Pneumonia, Viral/drug therapy , Pneumonia, Viral/pathology , SARS-CoV-2/isolation & purification
2.
Curr Opin Allergy Clin Immunol ; 21(6): 525-534, 2021 12 01.
Article in English | MEDLINE | ID: covidwho-1447630

ABSTRACT

PURPOSE OF REVIEW: The clinical outcomes from COVID-19 in monogenic causes of predominant antibody deficiency have pivotal implications for our understanding of the antiviral contribution of humoral immunity. This review summarizes the lessons learned from COVID-19 infection in X-linked agammaglobulinemia (XLA) due to genetic defects in Bruton's tyrosine kinase (BTK). RECENT FINDINGS: Key molecular pathways underlying the development of severe COVID-19 are emerging, highlighting the possible contribution of BTK to hyperinflammation. SARS-CoV-2 specific T-cell responses and complement activation appear insufficient to achieve viral clearance in some B-cell deficient individuals. Whilst appearing efficacious in this group, use of convalescent plasma has been recently associated with the evolution of viral escape variants. Early data suggests individuals with XLA can mount a viral-specific T-cell vaccine response, however, the clinical significance of this is still emerging. SUMMARY: In contrast to reports made early in the pandemic, we show XLA patients remain susceptible to severe disease. Persistent infection was common and is likely to carry a significant symptom burden and risk of novel variant evolution. COVID-19 infection in this vulnerable, antibody deficient group due to genetic, therapeutic or disease causes may require prompt and specific intervention for both patient and societal benefit.


Subject(s)
Agammaglobulinaemia Tyrosine Kinase/genetics , Agammaglobulinemia/complications , COVID-19/immunology , Genetic Diseases, X-Linked/complications , SARS-CoV-2/immunology , Agammaglobulinemia/genetics , Agammaglobulinemia/immunology , COVID-19/diagnosis , COVID-19/virology , Evolution, Molecular , Genetic Diseases, X-Linked/genetics , Genetic Diseases, X-Linked/immunology , Humans , SARS-CoV-2/genetics , SARS-CoV-2/pathogenicity , Severity of Illness Index
3.
Pediatr Allergy Immunol Pulmonol ; 34(3): 115-118, 2021 09.
Article in English | MEDLINE | ID: covidwho-1398070

ABSTRACT

Introduction: The Centers for Disease Control and Prevention (CDC) has listed primary immunodeficiency disorders as being predisposed to severe coronavirus disease 2019 (COVID-19). However, patients affected with X-linked agammaglobulinemia (XLA) have shown contrary results. In this study, we present 2 boys in late adolescence from south India with XLA who were infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), as well as a review of cases reported in the literature. Case Presentation: Two patients with XLA had been diagnosed late and were started on regular immunoglobulin prophylaxis only during adolescence. Both of them had developed bronchiectasis, an irreversible suppurative lung disease. However, both patients made an uneventful recovery without the need for artificial ventilation or convalescent plasma. Conclusion: Successful outcomes of patients with XLA and COVID-19, except for delayed recovery, from our experience and from global reports are intriguing and the role of B cell depletion is being studied as well. Further research and clinical experience are necessary to fully elucidate the reasons for these observations.


Subject(s)
Agammaglobulinemia/complications , COVID-19/physiopathology , Genetic Diseases, X-Linked/complications , Adolescent , COVID-19/complications , COVID-19/therapy , Humans , Male , SARS-CoV-2 , Young Adult
5.
Br J Haematol ; 195(2): 186-193, 2021 10.
Article in English | MEDLINE | ID: covidwho-1289897

ABSTRACT

Multiple myeloma (MM) patients are at excess risk for clinically significant COVID19 infection. BNT162b2 mRNA COVID19 (BNT162b2) vaccine provides effective protection against COVID19 for the general population, yet its effect in MM patients may be compromised due to disease and therapy-related factors and was not yet evaluated. This single-centre prospective study included MM patients tested for serological response 14-21 days post second vaccine. Vaccinated healthy volunteers served as controls. In all, 171 MM patients, median age 70 (38-94) were included; 159 active MM and 12 smouldering myeloma (SMM). Seropositive response rate (median titer) was 76% (91 U/ml) in active MM patients vs 98% (992 U/ml) in the 64 controls (P < 0·0001), and 100% (822 U/ml) in SMM patients. Multivariate analysis revealed older age (P = 0·009), exposure to ≥4 novel anti-myeloma drugs (P = 0·02) and hypogammaglobulinaemia (P = 0·002) were associated with lower response rates. None of the novel agents significantly decreased response rate, whereas daratumumab trended towards reduced response (P = 0·08). Adverse events occurred in 53% and 55% of the MM patients and controls, respectively, all transient grade 1-2. In conclusion, BNT162b2 vaccine was safe and provided a high seropositivity rate in MM patients, independent of treatment type. Older, hypogammaglobulinaemic and heavily pretreated patients had lower response rates.


Subject(s)
/adverse effects , COVID-19 Vaccines/adverse effects , COVID-19/prevention & control , Immunity, Humoral/immunology , Multiple Myeloma/immunology , Adult , Agammaglobulinemia/complications , Aged , Aged, 80 and over , Antibodies, Monoclonal/adverse effects , /immunology , COVID-19/diagnosis , COVID-19/epidemiology , COVID-19/immunology , COVID-19 Vaccines/administration & dosage , COVID-19 Vaccines/immunology , Case-Control Studies , Female , Humans , Immunity, Humoral/drug effects , Male , Middle Aged , Multiple Myeloma/complications , Multiple Myeloma/diagnosis , Multivariate Analysis , Predictive Value of Tests , Prospective Studies , Risk Factors , SARS-CoV-2/genetics , Treatment Outcome
6.
BMJ Case Rep ; 14(3)2021 Mar 04.
Article in English | MEDLINE | ID: covidwho-1136066

ABSTRACT

This report highlights the case of a patient with X-linked agammaglobulinaemia (XLA) and resultant bronchiectasis who was discharged from hospital after recovering from real-time reverse transcriptase-PCR positive COVID-19 infection having had a subsequent negative swab and resolution of symptoms, but was readmitted 3 weeks later with recrudescent symptoms and a further positive swab. Although there are reports of COVID-19 infection in XLA, for the first time we report a case of possible reinfection. Lessons learnt from this case include the potential for reinfection of COVID-19 in a patient with a weakened immune system and the importance of repeating COVID-19 swabs in inpatients. Extra caution needs to be taken when providing care in groups of patients who have a weakened or absent immune system.


Subject(s)
Agammaglobulinemia/complications , COVID-19/diagnosis , Genetic Diseases, X-Linked/complications , Reinfection/diagnosis , Reinfection/virology , Agammaglobulinemia/drug therapy , Anti-Bacterial Agents/therapeutic use , Anticoagulants/therapeutic use , Bronchiectasis/complications , COVID-19/complications , COVID-19/drug therapy , COVID-19 Nucleic Acid Testing/methods , Dexamethasone/therapeutic use , Fatal Outcome , Genetic Diseases, X-Linked/drug therapy , Humans , Male , Middle Aged , SARS-CoV-2/isolation & purification
7.
BMJ Case Rep ; 14(3)2021 Mar 04.
Article in English | MEDLINE | ID: covidwho-1119289

ABSTRACT

This report highlights the case of a patient with X-linked agammaglobulinaemia (XLA) and resultant bronchiectasis who was discharged from hospital after recovering from real-time reverse transcriptase-PCR positive COVID-19 infection having had a subsequent negative swab and resolution of symptoms, but was readmitted 3 weeks later with recrudescent symptoms and a further positive swab. Although there are reports of COVID-19 infection in XLA, for the first time we report a case of possible reinfection. Lessons learnt from this case include the potential for reinfection of COVID-19 in a patient with a weakened immune system and the importance of repeating COVID-19 swabs in inpatients. Extra caution needs to be taken when providing care in groups of patients who have a weakened or absent immune system.


Subject(s)
Agammaglobulinemia/complications , COVID-19/diagnosis , Genetic Diseases, X-Linked/complications , Reinfection/diagnosis , Reinfection/virology , Agammaglobulinemia/drug therapy , Anti-Bacterial Agents/therapeutic use , Anticoagulants/therapeutic use , Bronchiectasis/complications , COVID-19/complications , COVID-19/drug therapy , COVID-19 Nucleic Acid Testing/methods , Dexamethasone/therapeutic use , Fatal Outcome , Genetic Diseases, X-Linked/drug therapy , Humans , Male , Middle Aged , SARS-CoV-2/isolation & purification
9.
J Infect Dev Ctries ; 14(11): 1248-1251, 2020 11 30.
Article in English | MEDLINE | ID: covidwho-968244

ABSTRACT

We present atypical course of the novel coronavirus disease (COVID-19) in 34-year man with Bruton agammaglobulinemia. The patient was successfully treated by a combination of available drugs, including convalescent plasma and interleukin-6 (IL-6) inhibitor.


Subject(s)
Agammaglobulinemia/complications , COVID-19/complications , Adult , Agammaglobulinaemia Tyrosine Kinase , Agammaglobulinemia/drug therapy , COVID-19/drug therapy , Disease Progression , Humans , Immunoglobulins, Intravenous/administration & dosage , Male , SARS-CoV-2
12.
Pediatr Allergy Immunol ; 31(5): 565-569, 2020 07.
Article in English | MEDLINE | ID: covidwho-102307

ABSTRACT

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.


Subject(s)
Agammaglobulinemia/complications , Betacoronavirus , Coronavirus Infections/complications , Coronavirus Infections/drug therapy , Genetic Diseases, X-Linked/complications , Pneumonia, Viral/complications , Pneumonia, Viral/drug therapy , Adult , Agammaglobulinemia/therapy , Anti-Bacterial Agents/therapeutic use , COVID-19 , Enzyme Inhibitors/therapeutic use , Genetic Diseases, X-Linked/therapy , Humans , Hydroxychloroquine/therapeutic use , Immunization, Passive/methods , Italy , Male , Pandemics , SARS-CoV-2 , Treatment Outcome
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