Antibody deficiency testing for primary immunodeficiency

Marsh, Rebecca

doi:10.1016/j.anai.2019.08.012

Cited by 32 publications

(13 citation statements)

References 59 publications

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“…In the present study, IgG < 7.00 g/L occurred in 39% of patients with subnormal IgG1 only and 60% of patients with combined subnormal IgG1/IgG3. These latter observations are consistent with two respective reports that IgG was subnormal (< 7.00 g/L) in 38% and 46% of adults with IgGSD who had subnormal IgG1 only without other subnormal IgG subclasses, subnormal IgA, or subnormal IgM [5,6], contradicting previous suggestions that patients with low IgG1 will most likely be identified without IgG subclass testing [31,32].…”

Section: Discussionsupporting

confidence: 89%

Factors associated with IgG levels in adults with IgG subclass deficiency

Barton

Barton²,

Bertoli³

et al. 2021

BMC Immunol

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Background Factors associated with IgG levels in adults with IgG subclass deficiency (IgGSD) are incompletely understood. We studied adults with IgGSD with subnormal IgG1 only, subnormal IgG1/IgG3, or subnormal IgG3 only without other subnormal IgG subclasses, IgA, or IgM. We compiled: age; sex; autoimmune condition(s) (AC); atopy; IgG, IgG subclasses, IgA, IgM; IgGsum (IgG1 + IgG2 + IgG3 + IgG4); and D (percentage difference between IgGsum and IgG). We compared attributes of patients with/without subnormal IgG (< 7.00 g/L; subnormal IgG1 subclass groups only) and analyzed IgGsum and IgG relationships. We performed backward stepwise regressions on IgG using independent variables IgG subclasses, age, and sex and on D using independent variables age and sex. Results There were 39 patients with subnormal IgG1 only (89.7% women), 53 with subnormal IgG1/IgG3 (88.7% women), and 115 with subnormal IgG3 only (91.3% women). Fifteen patients (38.5%) and 32 patients (60.4%) in the respective subnormal IgG1 subclass groups had subnormal IgG. Attributes of patients with/without IgG < 7.00 g/L were similar, except that AC prevalence was lower in patients with subnormal IgG1 only and IgG < 7.00 g/L than ≥ 7.00 g/L (p = 0.0484). Mean/median IgG1 and IgG2 were significantly lower in patients with IgG < 7.00 g/L in both subnormal IgG1 subclass groups (p < 0.0001, all comparisons). Regressions on IgG in three subclass groups revealed positive associations with IgG1 and IgG2 (p < 0.0001 each association). Regressions on D revealed no significant association. IgG1 percentages of IgGsum were lower and IgG2 percentages were higher in patients with subnormal IgG1 subclass levels than subnormal IgG3 only (p < 0.0001 all comparisons). Conclusions We conclude that both IgG1 and IgG2 are major determinants of IgG in patients with subnormal IgG1, combined subnormal IgG1/IgG3, or subnormal IgG3 and that in patients with subnormal IgG1 or combined subnormal IgG1/IgG3, median IgG2 levels are significantly lower in those with IgG < 7.00 g/L than those with IgG ≥ 7.00 g/L.

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Section: Discussionsupporting

confidence: 89%

Factors associated with IgG levels in adults with IgG subclass deficiency

Barton

Barton²,

Bertoli³

et al. 2021

BMC Immunol

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“…IgG, IgA, and IgM levels were assessed with respect to age‐matched reference intervals. Protective titers to tetanus, diphtheria, or pneumococcal vaccines were determined by UCSF Clinical Immunology Laboratory with a standardized cut‐off 15 . Pre‐transplant testing for recipient included CMV IgG and RT‐PCR within 30 days prior to HSCT.…”

Section: Methodsmentioning

confidence: 99%

Unknown cytomegalovirus serostatus in primary immunodeficiency disorders: A new category of transplant recipients

Forlanini

Dara

Dvorak

et al. 2020

Transplant Infectious Dis

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Background Cytomegalovirus (CMV) serostatus of recipient (R) and donor (D) influences hematopoietic stem cell transplant (HSCT) outcome. However, it is not a reliable indicator of CMV infection in primary immunodeficiency disorder (PIDD) recipients who are unable to make adequate antigen‐specific immunoglobulin (Ig) or who receive intravenous Ig (IVIg) prior to testing. Objective Since no data exist on PIDD with unknown CMV serostatus, we aimed to evaluate the relationship between pre‐HSCT recipient and donor serostatus and incidence of CMV infection in recipients with unknown serostatus. Methods A retrospective analysis of all pediatric PIDD HSCTs (2007‐2018) was performed at University of California San Francisco. Recipients were separated into categories based on pre‐transplant serostatus: 1) seropositive (R(+)), 2) seronegative (R(‐)), and 3) unknown serostatus (R(x)). Patients with pre‐HSCT active CMV viremia were excluded. Results A total of 90 patients were included, 69% male. The overall incidence of CMV infection was 20%, but varied in R(+), R(‐), and R(x) at 80%, 0%, and 14%, (P‐value = .0001). Similarly, 5‐year survival differed among groups, 60% R(+), 100% R(‐), and 90% of R(x) (P‐value = .0045). There was no difference in CMV reactivation by donor serostatus (P‐value = .29), however, faster time to clearance of CMV was observed for R(x)/D(+) group (median 9.5 days (IQR 2.5‐18), P‐value = .024). Conclusion We identify a novel group of recipients, R(x), with an intermediate level of survival and CMV incidence post‐HSCT, when compared to seropositive and seronegative recipients. No evidence of CMV transmission from D(+) in R(‐) and R(x) was observed. We believe R(x) should be considered as a separate category in future studies to better delineate recipient risk status.

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“…Tetanus and diphtheria titers above 0.1 and up to 0.2 IU/mL, respectively, are considered protective, and seroconversion rates approach 100% one month after the second or third dose ( 60 , 61 ). Moreover, low levels after a vaccine booster in adult patients who have not been vaccinated for several years are expected, but children who have recently received routine immunization are expected to present a prominent response ( 62 ). Therefore, immunization records are crucial for interpreting vaccine responses.…”

Section: Predominantly Antibody Deficiencymentioning

confidence: 99%

A Critical Review on the Standardization and Quality Assessment of Nonfunctional Laboratory Tests Frequently Used to Identify Inborn Errors of Immunity

et al. 2021

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Inborn errors of immunity (IEI), which were previously termed primary immunodeficiency diseases, represent a large and growing heterogeneous group of diseases that are mostly monogenic. In addition to increased susceptibility to infections, other clinical phenotypes have recently been associated with IEI, such as autoimmune disorders, severe allergies, autoinflammatory disorders, benign lymphoproliferative diseases, and malignant manifestations. The IUIS 2019 classification comprises 430 distinct defects that, although rare individually, represent a group affecting a significant number of patients, with an overall prevalence of 1:1,200-2,000 in the general population. Early IEI diagnosis is critical for appropriate therapy and genetic counseling, however, this process is deeply dependent on accurate laboratory tests. Despite the striking importance of laboratory data for clinical immunologists, several IEI-relevant immunoassays still lack standardization, including standardized protocols, reference materials, and external quality assessment programs. Moreover, well-established reference values mostly remain to be determined, especially for early ages, when the most severe conditions manifest and diagnosis is critical for patient survival. In this article, we intend to approach the issue of standardization and quality control of the nonfunctional diagnostic tests used for IEI, focusing on those frequently utilized in clinical practice. Herein, we will focus on discussing the issues of nonfunctional immunoassays (flow cytometry, enzyme-linked immunosorbent assays, and turbidimetry/nephelometry, among others), as defined by the pure quantification of proteins or cell subsets without cell activation or cell culture-based methods.

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Antibody deficiency testing for primary immunodeficiency

Cited by 32 publications

References 59 publications

Factors associated with IgG levels in adults with IgG subclass deficiency

Factors associated with IgG levels in adults with IgG subclass deficiency

Unknown cytomegalovirus serostatus in primary immunodeficiency disorders: A new category of transplant recipients

A Critical Review on the Standardization and Quality Assessment of Nonfunctional Laboratory Tests Frequently Used to Identify Inborn Errors of Immunity

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