10 Warning Signs of Primary Immunodeficiency

1 Four or more new ear infections within 1 year.
2 Two or more serious sinus infections within 1 year.
3 Two or more months on antibiotics with little effect.
4 Two or more pneumonias within 1 year.
5 Failure of an infant to gain weight or grow normally.
6 Recurrent, deep skin or organ abscesses.
7 Persistent thrush in mouth or fungal infection on skin.
8 Need for intravenous antibiotics to clear infections.
9 Two or more deep-seated infections including septicemia.
10 A family history of PI.


10 Warning Signs of Primary Immunodeficiency For Adults

1 Two or more new ear infections within 1 year.
2 Two or more new sinus infections within 1 year, in the absence of allergy.
3 One pneumonia per year for more than 1 year.
4 Chronic diarrhea with weight loss.
5 Recurrent viral infections (colds, herpes, warts, condyloma).
6 Recurrent need for intravenous antibiotics to clear infections.
7 Recurrent, deep abscesses of the skin or internal organs.
8 Persistent thrush or fungal infection on skin or elsewhere.
9 Infection with normally harmless tuberculosis-like bacteria.
10 A family history of PI.


4 Stages of Testing for Primary Immunodeficiency

Stage 1
• History and physical examination, height and weight
• CBC and differential
• Quantitative Immunoglobulin levels lgG, lgM, IgA (related to age)

Stage 2
• Specific antibody responses (tetanus, diphtheria)
• Response to pneumococcal vaccine (pre/post) (for ages 3 and up)
• IgG subclass analysis

Stage 3
• Candida and Tetanus skin tests
• Lymphocyte surface markers CD3/CD4/CD8/CD19/CD16/CD56
• Mononuclear lymphocyte proliferation studies(using mitogen and antigen stimulation)
• Neutrophil oxidation burst (if indicated)

Stage 4
• Complement screening CH50, C3, C4
• Enzyme measurements adenosine deaminase, purine nucleoside phosphorylase)
• Phagocyte studies (surface glycoproteins, mobility, phagocytosis)
• NK cytotoxicity studies
• Further complement studies AH50
• Neo antigen to test antibody production
• Other surface/cytoplasmic molecules
• Cytokine receptor studies
• Family/genetic studies

Reference
http://info4pi.org/