Table 2.
Results of Immune Function Testing.
| Patient 1 | Patient 2 | Patient 3 | ||||||
| Initial | After 9 mo | Initial | After 12 mo | Initial | After 4 mo | Reference | ||
| Immunoglobulins | ||||||||
| IgG | 485 | 579 | 507 | 609 | 335–975 mg/dl | |||
| IgA | 25 | 40 | 91 | 40 | 17–70 mg/dl | |||
| IgM | 102 | 138 | 60 | 51 | 22–124 mg-31 | |||
| IgE | 11 | 8 | 37 | 0–17 IU/ml | ||||
| IgG subclasses | ||||||||
| IgG1 | 358 | 289 | 453 | 313–961 mg/dl | ||||
| IgG2 | 56 | 125 | 88 | 65–456 mg/dl | ||||
| IgG3 | 46 | 66 | 38 | 11–125 mg/dl | ||||
| IgG4 | 25 | 27 | 31 | 1–70 mg/dl | ||||
| Responses to vaccination | ||||||||
| Diphtheria antibodies | >5.00 | 0.06 | >0.10 IU/ml | |||||
| Tetanus antibodies | 1.74 | 0.10 | >0.10 IU/ml | |||||
| Haemophilus influenzae B | >22.0 | >2.0 µg/ml | ||||||
| Influenza A antibodies | >1:8 | 1:16 | >1:8 | <1:8 | ||||
| Influenza B antibodies | >1:8 | >1:8 | <1:8 | |||||
| Pneumococcal antibodies | 5/12 | 6/12 | 0/12 | 6/12 | Protective antibodies for 12 serotypes | |||
| Cell surface markers | ||||||||
| CD3 (%) | 57 | 46 | 21 | 19 | 59–87 | |||
| CD3 absolute | 2.993 | 0.676 | 0.504 | 0.416 | 1.180–6.960 × 109/l | |||
| CD3+CD4+(%) | 39 | 29 | 19 | 16 | 29–57 | |||
| CD3+CD4+absolute | 2.047 | 0.426 | 0.456 | 0.350 | 0.650–3.650 × 109/l | |||
| CD3+CD8+(%) | 16 | 15 | 2 | 2 | 7–31 | |||
| CD3+CD8+absolute | 0.840 | 0.221 | 0.048 | 0.044 | 0.160–1.980 × 109/l | |||
| CD4/+CD8+ratio | 2.44 | 1.93 | 9.50 | 8 | 1.00–3.50 | |||
| CD45 (%) | 100 | 100 | 100 | 100 | ||||
| CD3-CD16+CD56+(%) | 17 | 16 | 26 | 26 | 0–18 | |||
| CD3-CD16+CD56+absol | 0.893 | 0.235 | 0.624 | 0.569 | 0.000–1.440 | |||
| CD19 (%) | 25 | 37 | 53 | 54 | 6–19 | |||
| CD19 absolute | 1.313 | 0.544 | 1.272 | 1.183 | 0.150–1.520 × 109/l | |||
| Mitogen stimulation | ||||||||
| Mitogen control | 1893 | 14,058 | 863 | >50 | ||||
| Phytohemagglutinin | 271,852 | 95,118 | 388,752 | >188,880 | ||||
| Pokeweed | 184,725 | 45,415 | 109,556 | >68,549 | ||||
| Concanavalin A | 288,284 | 51,333 | 248,444 | >81,283 | ||||
| Staph A Cowen strain | 61,553 | 15,706 | 29,939 | >3,412 | ||||
| Antigen stimulation | ||||||||
| Candida | 0.4 | >2.0 (response ratio) | ||||||
| Streptomycin | 0.8 | >2.0 (response ratio) | ||||||
Bold typeface numbers = clinically relevant abnormal values.
Patient 1: Low IgG2 levels. Poor response to influenza vaccination. Patient 2: Low IgG2 levels. Poor response to protein antigens with non-protective diphtheria and tetanus antibody titers after immunization. Loss of protective antibodies against polysaccharide antigens, as evidenced by non-detectable pneumococcal antibodies following immunization. Low CD4+ T-cells. Poor T-cell proliferation demonstrated by impaired response to phytohemagglutinin, concanavalin A and pokeweed. Patient 3: Poor response to influenza vaccination. Low T-cell counts and low CD4+ and CD8+ T-cells. Poor lymphocyte proliferation following antigen stimulation.