Clinical summaries of patients with XIAP deficiency reported to date
| . | Rigaud et al,2 no. (%) . | Zhao et al,50 no. (%) . | Marsh et al,51 no. (%) . | Total, no. (%) . |
|---|---|---|---|---|
| No. of patients | 12 | 1 | 10 | 23 |
| Patients with HLH | 11 (92) | 1 | 9 (90) | 21 (91) |
| Patients with recurrent HLH or HLH-like illness | 6 (50) | 1 | 6 (60) | 13 (57) |
| Patients with EBV infection-associated HLH | 8 (67) | 1 | 3 (30) | 12 (52) |
| Patients with splenomegaly | 9 (75) | 1 | 9 (90) | 19 (83) |
| Patients with hypogammaglobulinema | 4 (33) | 0 | 2 (20) | 6 (26) |
| Patients with lymphoma | 0 | 0 | 0 | 0 (0) |
| Patients with colitis | 2 | 0 | 0 | 2 (9) |
| . | Rigaud et al,2 no. (%) . | Zhao et al,50 no. (%) . | Marsh et al,51 no. (%) . | Total, no. (%) . |
|---|---|---|---|---|
| No. of patients | 12 | 1 | 10 | 23 |
| Patients with HLH | 11 (92) | 1 | 9 (90) | 21 (91) |
| Patients with recurrent HLH or HLH-like illness | 6 (50) | 1 | 6 (60) | 13 (57) |
| Patients with EBV infection-associated HLH | 8 (67) | 1 | 3 (30) | 12 (52) |
| Patients with splenomegaly | 9 (75) | 1 | 9 (90) | 19 (83) |
| Patients with hypogammaglobulinema | 4 (33) | 0 | 2 (20) | 6 (26) |
| Patients with lymphoma | 0 | 0 | 0 | 0 (0) |
| Patients with colitis | 2 | 0 | 0 | 2 (9) |