Test Code VIRID Viral Susceptibility, Defects in Intrinsic and Innate Immunity, Gene Panel, Varies
Ordering Guidance
For patients with Epstein-Barr virus (EBV) susceptibility or a heritable predisposition to lymphoproliferative diseases, see EBLPD / Epstein-Barr Virus (EBV) Susceptibility and Lymphoproliferative Disorders Gene Panel, Varies.
Targeted testing for familial variants (also called site-specific or known variants testing) is available for the genes on this panel. See FMTT / Familial Variant, Targeted Testing, Varies. To obtain more information about testing option, call 800-533-1710.
Shipping Instructions
Specimen preferred to arrive within 96 hours of collection.
Specimen Required
Patient Preparation: A previous bone marrow transplant from an allogenic donor will interfere with testing. Call 800-533-1710 for instructions for testing patients who have received a bone marrow transplant.
Submit only 1 of the following specimens:
Specimen Type: Whole blood
Container/Tube:
Preferred: Lavender top (EDTA) or yellow top (ACD)
Acceptable: Any anticoagulant
Specimen Volume: 3 mL
Collection Instructions:
1. Invert several times to mix blood.
2. Send whole blood specimen in original tube. Do not aliquot.
Specimen Stability Information: Ambient (preferred) 4 days/Refrigerated
Specimen Type: Skin biopsy
Supplies: Fibroblast Biopsy Transport Media (T115)
Container/Tube: Sterile container with any standard cell culture media (eg, minimal essential media, RPMI 1640). The solution should be supplemented with 1% penicillin and streptomycin.
Specimen Volume: 4-mm punch
Specimen Stability Information: Refrigerated (preferred)/Ambient
Additional Information: A separate culture charge will be assessed under CULFB /Fibroblast Culture for Biochemical or Molecular Testing. An additional 3 to 4 weeks is required to culture fibroblasts before genetic testing can occur.
Specimen Type: Cultured fibroblasts
Container/Tube: T-25 flask
Specimen Volume: 2 Flasks
Collection Instructions: Submit confluent cultured fibroblast cells from a skin biopsy from another laboratory. Cultured cells from a prenatal specimen will not be accepted.
Specimen Stability Information: Ambient (preferred)/Refrigerated (<24 hours)
Additional Information: A separate culture charge will be assessed under CULFB /Fibroblast Culture for Biochemical or Molecular Testing. An additional 3 to 4 weeks is required to culture fibroblasts before genetic testing can occur.
Forms
1. New York Clients-Informed consent is required. Document on the request form or electronic order that a copy is on file. The following documents are available:
-Informed Consent for Genetic Testing (T576)
-Informed Consent for Genetic Testing (Spanish) (T826)
2. Molecular Genetics: Congenital Inherited Diseases Patient Information (T521)
Secondary ID
619900Useful For
Providing a comprehensive genetic evaluation for patients with a personal or family history suggestive of an inborn error of immunity causing a hereditary form of severe viral susceptibility
Establishing a diagnosis of hereditary form of viral susceptibility, allowing for appropriate management and surveillance for disease features based on the gene and/or variant involved
Identifying variants within genes known to be associated with a hereditary form of viral susceptibility, allowing for predictive testing of at-risk family members
Reflex Tests
Test ID | Reporting Name | Available Separately | Always Performed |
---|---|---|---|
CULFB | Fibroblast Culture for Genetic Test | Yes | No |
Testing Algorithm
For skin biopsy or cultured fibroblast specimens, fibroblast culture will be performed at an additional charge. If viable cells are not obtained, the client will be notified.
Special Instructions
- Informed Consent for Genetic Testing
- Informed Consent for Genetic Testing (Spanish)
- Targeted Genes and Methodology Details for Viral Susceptibility, Defects in Intrinsic and Innate Immunity, Gene Panel
- Viral Susceptibility, Lymphoproliferation, and Hemophagocytic Lymphohistiocytosis Patient Information
Method Name
Sequence Capture and Targeted Next-Generation Sequencing (NGS)
Reporting Name
Viral Susceptibility Gene PanelSpecimen Type
VariesSpecimen Minimum Volume
Blood: 1 mL; Skin biopsy or cultured fibroblasts: See Specimen Required
Specimen Stability Information
Specimen Type | Temperature | Time | Special Container |
---|---|---|---|
Varies | Varies |
Reject Due To
All specimens will be evaluated at Mayo Clinic Laboratories for test suitability.Clinical Information
Viral infections are common in otherwise healthy individuals, but they may also present clinically due to a primary (genetic) immunodeficiency (ie, inborn error of immunity: IEI). Alternatively, secondary immunodeficiencies may have a similar presentation but result from immunosuppressive medication or illness, such as HIV infection. IEIs may cause a susceptibility to an entire group of pathogens (bacteria, fungi, or viruses), a subset of pathogens (eg, RNA viruses), or can cause susceptibility specific to a single pathogen (eg, Epstein-Barr virus [EBV], human papillomavirus [HPV]). IEIs may also lead to a more severe presentation, including fatal infection caused by a pathogen that usually causes only a mild or non-fatal disease (eg, influenza). This panel targets IEIs that lead to susceptibility to viruses or to a subset of viruses, severe viral pneumonia, or a specific virus. Examples of infections where this gene panel is useful include EBV, skin-tropic beta-HPV, influenza, and SARS-CoV-2. IEIs that lead to systemic immune deficiencies and susceptibility to a large variety of pathogens (eg, T-cell deficiencies) are not included in this panel.
EBV is the cause of infectious mononucleosis and persists asymptomatically for life in nearly all adults. It is also associated with the development of T- and B-cell lymphomas, nasopharyngeal and gastric carcinomas, and other malignancies. EBV infection in IEIs can present with fulminant infectious mononucleosis, hemophagocytosis, B-cell proliferative disease (including lymphoma), and hypogammaglobulinemia.
Beta-HPVs circulate silently in the general population and cause no visible lesions in most people. Genetic susceptibility to beta-HPVs leads to warts, pityriasis-like lesions, epidermodysplasia verruciformis, and increased risk of non-melanoma skin cancers.
Seasonal influenza viruses are common RNA viruses that infect the respiratory tract, causing a benign illness in most infected individuals. Influenza pneumonia or acute respiratory distress syndrome are rare, and the case fatality ratio is less than 1%. Children with severe influenza have been found to carry defects in IRF7, IRF9, STAT1, STAT2, and TLR3. Similarly, the COVID-19 pandemic has revealed that SARS-CoV-2 infection can lead to asymptomatic infection as well as fatal pneumonia. Genetic studies showed that approximately 2 to 3% of cases of severe life-threatening SARS-CoV-2 infection resulted from IEI, mainly genetic defects in the TLR3- or TLR7-dependent type 1 interferon pathway (eg, TLR3, TLR7, IFNAR1/2, STAT2, and IRF7), overlapping with that of severe pneumonia susceptibility in influenza infections.
Reference Values
An interpretive report will be provided.
Interpretation
All detected variants are evaluated according to American College of Medical Genetics and Genomics recommendations.(1) Variants are classified based on known, predicted, or possible pathogenicity and reported with interpretive comments detailing their potential or known significance.
Method Description
Next-generation sequencing (NGS) and/or Sanger sequencing are performed to test for the presence of variants in coding regions and intron/exon boundaries of the genes analyzed, as well as some other regions that have known disease-causing variants. The human genome reference GRCh37/hg19 build was used for sequence read alignment. At least 99% of the bases are covered at a read depth over 30X. Sensitivity is estimated at above 99% for single nucleotide variants, above 94% for deletions/insertions (delins) less than 40 base pairs (bp), and above 95% for deletions up to 75 bp and insertions up to 47 bp. NGS and/or a polymerase chain reaction-based quantitative method is performed to test for the presence of deletions and duplications in the genes analyzed.
There may be regions of genes that cannot be effectively evaluated by sequencing or deletion and duplication analysis as a result of technical limitations of the assay, including regions of homology, high guanine-cytosine (GC) content, and repetitive sequences. See Targeted Genes and Methodology Details for Viral Susceptibility, Defects in Intrinsic and Innate Immunity, Gene Panel for details regarding the targeted genes analyzed for each test and specific gene regions not routinely covered.(Unpublished Mayo method)
Confirmation of select reportable variants may be performed by alternate methodologies based on internal laboratory criteria.
Genes analyzed: CARMIL2, CD27, CD70, CIB1, CTPS1, CXCR4, DBR1, IFIH1, IFNAR1, IFNAR2, IRF3, IRF7, IRF9, MAGT1, POLR3A, POLR3C, PRKCD, RASGRP1, SH2D1A, STAT1, STAT2, TLR3, TLR7, TLR8, TMC6, TMC8, TNFRSF9, TRAF3, UNC93B1, and XIAP
Day(s) Performed
Varies
Performing Laboratory
Mayo Clinic Laboratories in RochesterTest Classification
This test was developed and its performance characteristics determined by Mayo Clinic in a manner consistent with CLIA requirements. It has not been cleared or approved by the US Food and Drug Administration.CPT Code Information
81443
88233-Tissue culture, skin, solid tissue biopsy (if appropriate)
88240-Cryopreservation (if appropriate)
LOINC Code Information
Test ID | Test Order Name | Order LOINC Value |
---|---|---|
VIRID | Viral Susceptibility Gene Panel | 103742-3 |
Result ID | Test Result Name | Result LOINC Value |
---|---|---|
619901 | Test Description | 62364-5 |
619902 | Specimen | 31208-2 |
619903 | Source | 31208-2 |
619904 | Result Summary | 50397-9 |
619905 | Result | 82939-0 |
619906 | Interpretation | 69047-9 |
619907 | Additional Results | 82939-0 |
619908 | Resources | 99622-3 |
619909 | Additional Information | 48767-8 |
619910 | Method | 85069-3 |
619911 | Genes Analyzed | 82939-0 |
619912 | Disclaimer | 62364-5 |
619913 | Released By | 18771-6 |