Characteristics of EBV-positive and EBV-negative XLP1 patients
| . | EBV positive (64.6%, n = 51) . | EBV negative (35.4%, n = 28) . |
|---|---|---|
| Median age at presentation | 4 y (8 mo-40 y) | 3 y (0-31 y) |
| Family history of XLP1 | 17.6% | 42.9% |
| HLH | 51% | 21.4% |
| FIM | 17.6% | |
| Lymphoma | 19.6% | 25% |
| Dysgammaglobulinemia | 37.2% | 51.8% |
| Mortality | 35.2% | 28.6% |
| Median age at death | 3 y 6 mo (14 mo-21 y) | 5 y 11 mo (20 mo-31 y) |
| . | EBV positive (64.6%, n = 51) . | EBV negative (35.4%, n = 28) . |
|---|---|---|
| Median age at presentation | 4 y (8 mo-40 y) | 3 y (0-31 y) |
| Family history of XLP1 | 17.6% | 42.9% |
| HLH | 51% | 21.4% |
| FIM | 17.6% | |
| Lymphoma | 19.6% | 25% |
| Dysgammaglobulinemia | 37.2% | 51.8% |
| Mortality | 35.2% | 28.6% |
| Median age at death | 3 y 6 mo (14 mo-21 y) | 5 y 11 mo (20 mo-31 y) |