Table 1B.
Patient transplant characteristics - non-SCID.
| Variable | 1974–1989 | 1990–1999 | 2000–2009 | 2010–2016 |
|---|---|---|---|---|
| Number of patients | 36 | 319 | 731 | 816 |
| Disease sub-types | ||||
| HLH, XLP, CH | 8 (22) | 129 (40) | 381 (52) | 413 (51) |
| Wiskott-Aldrich syndrome | 19 (53) | 120 (38) | 145 (20) | 107 (13) |
| Chronic granulomatous disease | 0 | 11 (3) | 68 (9) | 159 (19) |
| Other Non-SCID PID | 9 (25) | 59 (19) | 137 (19) | 137 (17) |
| Age at transplant, years, Median (Inter-quartile range) | 4 (1–8) | 2 (1–5) | 2 (1–6) | 3 (1–10) |
| Donor Type | ||||
| HLA identical sibling | 16 (44) | 70 (22) | 144 (20) | 142 (17) |
| Other related donor | 10 (28) | 47 (15) | 42 (6) | 60 (7) |
| Matched other relative | 0 | 0 | 12 | 23 |
| Mismatched other relative | 0 | 0 | 30 | 37 |
| HLA-match not reported | 10 | 47 | 0 | 0 |
| Unrelated donor | 10 (28) | 202 (63) | 545 (75) | 614 (75) |
| Graft type | ||||
| Bone marrow | 36 | 256 (80) | 418 (57) | 531 (65) |
| Peripheral blood | 0 | 12 (4) | 68 (9) | 117 (14) |
| Cord blood | 0 | 51 (16) | 245 (34) | 168 (21) |
| Conditioning intensity | ||||
| Myeloablative | 32 (89) | 290 (91) | 579 (79) | 382 (47) |
| Reduced intensity | 4 (11) | 29 (9) | 152 (21) | 434 (53) |
| In-vivo T-cell depletion | ||||
| No | 28 (78) | 142 (45) | 210 (29) | 113 (14) |
| Yes | 8 (22) | 177 (55) | 521 (71) | 703 (86) |
| GVHD Prophylaxis | ||||
| Ex-vivo T-cell depletion | 6 (17) | 35 (11) | 33 (5) | 10 (1) |
| CD34+ selection | 0 | 0 | 15 (2) | 24 (3) |
| Cyclosporine +/− MMF/MTX | 14 (39) | 247 (77) | 533 (73) | 472 (58) |
| Tacrolimus +/− MMF/MTX | 0 | 5 (2) | 104 (14) | 232 (29) |
| Other | 16 (44) | 32 (10) | 46 (6) | 78 (10) |
HLH, Hemophagocytic Lymphohistiocytosis
XLP, X-linked Lymphoproliferative Disease
CH, Chediak-Higashi Syndrome
SCID, Severe Combined Immune Deficiency
PID, Primary Immune Deficiency
HLA, Human Leukocyte Antigen
GVHD, Graft Versus Host Disease
MMF, mycophenolate mofetil
MTX, methotrexate