Table 2.
Transplantation procedures and acute GVHD in the Japanese patients with XIAP deficiency
| Patient | ID | Graft HLA match | Graft source | Total cell count (cells/kg) | CD34+ cell count (cells/kg) | Conditioning regimen (mg/m2 or mg/kg) | ATG (mg/kg) | GVHD prophylaxis | Acute GVHD |
|---|---|---|---|---|---|---|---|---|---|
| 1 | 2.1 | 7/8 (C) | CB | 1.01 × 108 | 1.96 × 105 | Flu 125, Mel 80, CY 140, TBI 6Gy | – | MTX, Tac | |
| 2 | 8 | 8/8 | CB | 7.63 × 107 | 2.56 × 105 | Eto 300, Flu 180, Mel 140, TBI 4Gy | 5 | Tac | I (skin 1) |
| 3 | 1 | 8/8 | URBM | 3.46 × 108 | N/A | Flu 150, Mel 70, TLI 3Gy | 1.25 | MTX, Tac | III (gut 2, skin 3) |
| 4 | 11 | 7/8 (A) | URBM | 2.6 × 108 | 2.7 × 106 | Flu 150, Mel140, TBI 4Gy | – | MTX, Tac | I (skin 2) |
| 5 | 13 | 5/8 (A, C, DR) | CB | 4.4 × 107 | 1.1 × 105 | Flu 150, CY 120, TBI 4Gy | – | MTX, Tac | I (skin 2) |
| 6 | 5.2 | 8/8 | RBM | 1.85 × 108 | N/A | Eto 300, Flu 150, Mel 140, TBI 3Gy | – | MTX, Tac | I (skin 1) |
| 7 | 2.3 | 7/8 (C) | URBM | 5.8 × 108 | N/A | Flu 150, Mel 140, TBI 3Gy | – | MTX, Tac | – |
| 8 | 14 | 7/8 (DR) | CB | 1.14 × 108 | 1.49 × 105 | Eto 100, Flu 150, Mel 140, TLI 3Gy | – | MTX, Tac | I (skin 1) |
| 9 | 15.2 | 7/8 (DR) | URBM | 3.66 × 108 | N/A | Eto 200, Flu 150, Mel 140, TBI 4Gy | 5 | MTX, Tac | – |
| 10 | 18 | 8/8 | URBM | 1.43 × 108 | 1.73 × 106 | Eto 200, Flu 150, Mel 140, TBI 3Gy | 2.5 | MTX, Tac | I (skin 1) |
ATG antithymocyte globulin, GVHD graft versus host disease, CB cord blood, N/A not available, URBM unrelated bone marrow, RBM related bone marrow, Flu fludarabine, Mel melphalan, CY cyclophosphamide, TBI total body irradiation, Eto etoposide, TLI total lymphoid irradiation, MTX methotrexate, Tac tacrolimus