Table 1.
Clinical features of patients with and without SIEs according to diagnosis and treatment.
| Patients with at least 1 SIE (n = 13) | No SIEs (n = 83) | p | |
|---|---|---|---|
| Diagnosis | n (%) | n (%) | 0.038 |
| ALPS-like | 10 (76.9%) | 37 (44.6%) | |
| Other | |||
| ITP | 1 (7.7%) | 29 (34.9%) | |
| ALPS | 2 (15.4%) | 17 (20.5%) | |
| Therapy during follow up | n (%) | n (%) | 0.05 |
| Only MMF | 1 (7.7%) | 34 (41%) | |
| Only SR | 5 (38.5%) | 26 (31.3%) | |
| SR after MMF failure | 7 (53.8%) | 23 (27.7%) | |
| Therapy duration (months) | Median, IQR | Median, IQR | |
| Total | 54 (55) | 47 (59.5) | 0.337 |
| Mycophenolate | 20 (62.5) | 32.5 (53.8) | 0.925 |
| Sirolimus | 40 (46.8) | 23 (44) | 0.107 |
ALPS, autoimmune lymphoproliferative syndrome; IQR, interquartile range; MMF, mycophenolate mofetil; SIE, severe infectious event; SR, sirolimus.