The Jeffrey Modell Foundation created the 4 Stages of Testing Poster in the 1990s to assist physicians in making a Primary Immunodeficiency diagnosis. The poster has been translated into more than 50 languages to help diagnose patients across the world!
Stage 1
- History and physical examination
- CBC and differential
- Quantitative Immunoglobulin levels IgG, IgM, IgA 1
Stage 2
- Specific antibody responses (tetanus, diphtheria, pneumococcus)
- Lymphocyte surface markers CD3/CD4/CD8/CD19/CD56
Stage 3
- Lymphocyte proliferation studies (mitogen/antigen stimulation or skin delayed type hypersensitivity)
- Neutrophil oxidation burst (if indicated)
- Response to pneumococcal vaccine (for ages 3 and up)
- Primary Immunodeficiency gene sequencing panel
Stage 4
- Complement screening CH50, specific complement components, AH50
- Enzyme activity measurements (e.g., adenosine deaminase, purine nucleoside phosphorylase)
- Phagocyte studies (e.g., surface glycoproteins, mobility, phagocytosis)
- NK cytotoxicity studies
- Neo antigen response to test antibody production
- Other surface molecules for detailed immunophenotype (e.g., memory B cells, T/NK cell subpopulations)
- Specific protein levels (e.g., SAP, Perforin, WASp)
- Cytokine or other function receptor quantification
- IgG subclass analysis
- Genomic studies
To read the 4 Stages poster in additional languages, click here: http://info4pi.org/library/educational-materials/10-warning-signs-international.