Table 4.
Immunoglobulin (Ig) replacement therapy and hematopoietic stem cell transplant history among 231 patients with X-linked agammaglobulinemia from the USIDNET Registry
| Feature of therapy | N (%) or median (IQR) | |
|---|---|---|
| Ig therapy | Received | 187 (81.0%) |
| Did not receive | 1 (0.4%) | |
| Unknown or not reported | 43 (18.6%) | |
| Ig route (specified in 187 of 225 patients) | Intravenous | 83 (44.4%) |
| Subcutaneous | 45 (24.1%) | |
| Intramuscular | 1 (0.5%) | |
| Other | 2 (1.1%) | |
| Unknown/not reported | 56 (29.9%) | |
| Median age, years | Ig started | 1.8 (0.7–4.0) |
| Median age, years | Patients receiving intravenous Ig | 16.2 (8.2–25.0) |
| Patients receiving subcutaneous Ig | 15.6 (9.2–25.0) | |
| Median frequency, days | Intravenous Ig | 28 (21–30) |
| Subcutaneous Ig | 7 (7–14) | |
| Median dose, mg/kg | Intravenous Ig | 490 (351–699) |
| Subcutaneous Ig | 157 (121–192) | |
| Hematopoietic stem cell transplant, N (%) | Received | 4 (1.7%) |
| Did not receive | 121 (52.4%) | |
| Unknown or not reported | 106 (45.9%) |
Ig immunoglobulin, IQR interquartile ratio, USIDNET United States Immunodeficiency Network