Table 1. Humoral Immunophenotype for proband, sibling and father.
Immunoglobulin totals show consistent adolescent onset of CVID in the proband (P), brother (B), and father (F). Values for the father (F) prior to IVIG therapy were not available. Bolded values are abnormal, normal ranges are in parentheses. Values with an asterisk (*) denote levels while on immunoglobulin replacement.
| Patient | F | F | B | B | B | B | P | P | P | P |
|---|---|---|---|---|---|---|---|---|---|---|
| Age at analysis | 46 | 65 | 14 | 15 | 17 | 25 | 17 | 18 | 19 | 23 |
| IgG (mg/dl) | 559* (717–1411) | 1550* (717–1411) | 815 (717–1411) | 712 (600–1600) | 391 (600–1600) | 2030* (717–1411) | 926 (700–1600) | 687 (700–1600) | 620 (700–1600) | 1190* (635–1775) |
| IgG1 | 738 (315–855) | 582 (155–1020) | 313 (155–1020) | 767 (490–1140) | ||||||
| IgG2 | 17 (64–495) | 17 (44–495) | <12 (44–495) | 42 (150–640) | ||||||
| IgG3 | 42 (23–196) | 23 (8–209) | 19 (8–209) | 15 (11–85) | ||||||
| IgA (mg/dl) | <13 (78–391) | <7 (78–391) | <7 (78–391) | <5.9 (70–315) | <5.9 (70–315) | <7 (717–1411) | <7 (70–400) | <7 (70–400) | <6 (70–486) | |
| IgM (mg/dl) | 27 (53–334) | 27 (53–334) | 35 (53–334) | 22.4 (40–250) | 6.8 (40–250) | 12 (53–334) | <10 (40–230) | <10 (40–230) | <4 (71–237) |