Comparison of XLP-1 and XLP-2 phenotypes
| . | SAP-/Y, n (%) . | XIAP-/Y, n (%) . | P* . |
|---|---|---|---|
| HLH | 18 of 33 (55) | 22 of 29 (76) | NS |
| HLH relapses (/HLH-survivors) | 2 of 7 (29) | 11 of 14 (79) | NS |
| EBV at first HLH | 11 of 12 (92) | 15 of 18 (83) | NS |
| Fatal HLH | 11 of 33 (33) | 5 of 30 (17) | NS |
| Fatal HLH (/HLH patients) | 11 of 18 (61) | 5 of 22 (23) | .0230 |
| Hypogammaglobulinemia | 14 of 21 (67) | 8 of 24 (33) | .0377 |
| Lymphoma | 10 of 33 (30) | 0 of 30 (0) | .0010 |
| Cytopenias (in the absence of full-blown HLH) | 4 of 33 (12) | 11 of 21 (52) | .0020 |
| Splenomegaly (in the absence of full-blown HLH) | 2 of 29 (7) | 20 of 23 (87) | <.0001 |
| Hemorrhagic colitis | 0 of 33 (0) | 5 of 30 (17) | .0203 |
| . | SAP-/Y, n (%) . | XIAP-/Y, n (%) . | P* . |
|---|---|---|---|
| HLH | 18 of 33 (55) | 22 of 29 (76) | NS |
| HLH relapses (/HLH-survivors) | 2 of 7 (29) | 11 of 14 (79) | NS |
| EBV at first HLH | 11 of 12 (92) | 15 of 18 (83) | NS |
| Fatal HLH | 11 of 33 (33) | 5 of 30 (17) | NS |
| Fatal HLH (/HLH patients) | 11 of 18 (61) | 5 of 22 (23) | .0230 |
| Hypogammaglobulinemia | 14 of 21 (67) | 8 of 24 (33) | .0377 |
| Lymphoma | 10 of 33 (30) | 0 of 30 (0) | .0010 |
| Cytopenias (in the absence of full-blown HLH) | 4 of 33 (12) | 11 of 21 (52) | .0020 |
| Splenomegaly (in the absence of full-blown HLH) | 2 of 29 (7) | 20 of 23 (87) | <.0001 |
| Hemorrhagic colitis | 0 of 33 (0) | 5 of 30 (17) | .0203 |
Calculated with Fisher exact tests.