Table I.
Demographic, clinical and immunologic characteristics of four patients with severe HPV disease.
| Patient 1 | Patient 2 | Patient 3 | Patient 4 | |
|---|---|---|---|---|
| SCID Phenotype | T-B+NK− | T-B+NK− | T-B+NK− | T-B+NK− |
| Specific diagnosis | γc | Unknown | γc | γc |
| Age at transplant (days) | 22 | 120 | 14 | 219 |
| Type of Transplant | MMRD mother | MMRD mother | MRD sister | MRD sister |
| Pre-transplant conditioning | None | None | None | None |
| T cell Depletion | None | None | None | None |
| Engraftment | Mixed (T+B−) | Mixed (T+B−) | Mixed (T+B−) | Mixed (T+B−) |
| T cell Engraftment (%) | 100 | 99 | 98 | 100 |
| Time of onset of warts after transplant (years) | 7.75 | 9.4 | 7 | 5.75 |
| Location of warts | Face, trunk | Trunk, arms | Face, hands, feet, arms, legs | Face, hands, feet, arms, legs |
| Other Long Term Comorbidities | PLE, Asthma, On Immunoglobulin replacement | None | None | None |
| Most recent ALC cells/mm3 | 1755 | 2014 | 1650 | 1482 |
| Most recent CD3 cells/mm3 (%) | 1615 (92%) | 1672 (83%) | 1518 (92%) | 1289 (87%) |
| Most recent CD4 cells/mm3 (%) | 562 (32%) | 467 (23%) | 726 (45%) | 888 (60%) |
| Most recent CD19 cells/mm3 (%) | 123 (7%) | 282 (14%) | 99 (6%) | 133 (9%) |
| Most recent IgG mg/dl | 599 | 873 | 1139 | 1690 |
(MRD: Matched Related Transplant, MMRD: Mismatched related transplant, PLE: Protein Losing enteropathy, ALC: Absolute Lymphocyte count)