B-Cell Functions of 22 Patients With B+SCID After BMT
| . | UPN . | Diagnosis . | B-Cell Chimerism . | IVIG Treatment . | IgG (mg/mL) . | IgA (mg/mL) . | IgM (mg/mL) . | IgE (IU/mL) . | IgG Isotype . | Antibody Production . | CD3 (/mL) . | T-Cell Function . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| HLA-identical BMT | 17 | γc | H | Yes | / | 0.9 | 1 | ND | / | / | 2,300 | N |
| 50 | Unknown | 50%D | Yes | 20.4 | 1.1 | 1.3 | 1,474 | No IgG2 | Normal | 1,200 | Low | |
| 116* | γc | H | No | 8 | 1.4 | 1 | 37 | Normal | Normal | 1,960 | N | |
| 261 | γc | H | No | 8.6 | 0 | 1 | 0 | Low IgG2 | Normal | 4,950 | N | |
| 299 | Unknown | H | No | 8.8 | 0.9 | 1 | 14 | Normal | Normal | 2,300 | N | |
| HLA-nonidentical BMT | 51* | γc | H | Yes | / | 0 | 1.3 | ND | / | / | 760 | N |
| 168 | γc | H | Yes | / | 0 | 1.4 | ND | / | / | 3,290 | N | |
| 272b | γc | H | Yes | / | 0.2 (−) | 0.9 | ND | / | / | 1,000 | N | |
| 277 | γc | H | Yes | / | 0 | 0.4 (−) | 0 | / | / | 2,320 | N | |
| 303 | γc | H | Yes | / | 1 | 2.8 (+) | 48 | / | / | 1,920 | N | |
| 307 | γc | H | Yes | / | 1.3 | 3.2 (+) | ND | / | / | 320 | Low | |
| 308 | γc | H | Yes | / | 0.2 (−) | 0.8 | 3 | / | / | 4,500 | N | |
| 325 | γc | H | Yes | / | 0.5 (−) | 0.3 (−) | 3 | / | / | 2,288 | N | |
| 348 | γc | H | Yes | / | 0 | 0.4 | 0 | / | / | 2,520 | N | |
| 318 | γc | H | No | 8 | 0.8 | 4 (+) | 11 | ND | Dissociated† | 2,370 | N | |
| 122 | γc | D | No | 10.7 | 0.8 | 1.2 | 20 | Normal | Normal | 1,700 | N | |
| 158 | Unknown | H | No | 5.2 | 1 | 0.9 | ND | Low IgG2 | Normal | 784 | N | |
| 360 | Unknown | H | No | 13.9 | 0 | 2 (+) | ND | ND | Normal | 2,145 | N | |
| 57 | JAK3 | H | No | 11.2 | 0 | 2.7 (+) | ND | Normal | Dissociated‡ | 2,000 | N | |
| 221 | JAK3 | D | No | 7.6 | 1.2 | 0.7 | 22 | ND | Normal | 3,198 | N | |
| 223 | JAK3 | D | No | 10 | 0.4 (−) | 1.5 | 25 | Normal | Dissociated2-153 | 2,500 | N | |
| 365 | JAK3 | H | No | 7.7 | 0 | 2.1 (+) | 37 | Normal | Normal | 3,120 | N |
| . | UPN . | Diagnosis . | B-Cell Chimerism . | IVIG Treatment . | IgG (mg/mL) . | IgA (mg/mL) . | IgM (mg/mL) . | IgE (IU/mL) . | IgG Isotype . | Antibody Production . | CD3 (/mL) . | T-Cell Function . |
|---|---|---|---|---|---|---|---|---|---|---|---|---|
| HLA-identical BMT | 17 | γc | H | Yes | / | 0.9 | 1 | ND | / | / | 2,300 | N |
| 50 | Unknown | 50%D | Yes | 20.4 | 1.1 | 1.3 | 1,474 | No IgG2 | Normal | 1,200 | Low | |
| 116* | γc | H | No | 8 | 1.4 | 1 | 37 | Normal | Normal | 1,960 | N | |
| 261 | γc | H | No | 8.6 | 0 | 1 | 0 | Low IgG2 | Normal | 4,950 | N | |
| 299 | Unknown | H | No | 8.8 | 0.9 | 1 | 14 | Normal | Normal | 2,300 | N | |
| HLA-nonidentical BMT | 51* | γc | H | Yes | / | 0 | 1.3 | ND | / | / | 760 | N |
| 168 | γc | H | Yes | / | 0 | 1.4 | ND | / | / | 3,290 | N | |
| 272b | γc | H | Yes | / | 0.2 (−) | 0.9 | ND | / | / | 1,000 | N | |
| 277 | γc | H | Yes | / | 0 | 0.4 (−) | 0 | / | / | 2,320 | N | |
| 303 | γc | H | Yes | / | 1 | 2.8 (+) | 48 | / | / | 1,920 | N | |
| 307 | γc | H | Yes | / | 1.3 | 3.2 (+) | ND | / | / | 320 | Low | |
| 308 | γc | H | Yes | / | 0.2 (−) | 0.8 | 3 | / | / | 4,500 | N | |
| 325 | γc | H | Yes | / | 0.5 (−) | 0.3 (−) | 3 | / | / | 2,288 | N | |
| 348 | γc | H | Yes | / | 0 | 0.4 | 0 | / | / | 2,520 | N | |
| 318 | γc | H | No | 8 | 0.8 | 4 (+) | 11 | ND | Dissociated† | 2,370 | N | |
| 122 | γc | D | No | 10.7 | 0.8 | 1.2 | 20 | Normal | Normal | 1,700 | N | |
| 158 | Unknown | H | No | 5.2 | 1 | 0.9 | ND | Low IgG2 | Normal | 784 | N | |
| 360 | Unknown | H | No | 13.9 | 0 | 2 (+) | ND | ND | Normal | 2,145 | N | |
| 57 | JAK3 | H | No | 11.2 | 0 | 2.7 (+) | ND | Normal | Dissociated‡ | 2,000 | N | |
| 221 | JAK3 | D | No | 7.6 | 1.2 | 0.7 | 22 | ND | Normal | 3,198 | N | |
| 223 | JAK3 | D | No | 10 | 0.4 (−) | 1.5 | 25 | Normal | Dissociated2-153 | 2,500 | N | |
| 365 | JAK3 | H | No | 7.7 | 0 | 2.1 (+) | 37 | Normal | Normal | 3,120 | N |
Abbreviations: H, cells of host origin; D, cells of donor origin; ND, not done; (−) and (+), respectively, low or high level of Ig.
These 2 patients had the same γc gene mutation.
Normal production of antibodies against Tetanus and Diphtheria toxoids, Polio 1 and 3, but not Polio 2 antigen.
Normal production of antibodies against Diphtheria toxoid, Polio 1 and 2, but not Polio 3 and Tetanus toxoid.
Normal production of antibodies against Diphtheria toxoid, Polio 2 and 3, but not Polio 1 and Tetanus toxoid.