Characteristics of 170 patients receiving allogeneic BMT for Wiskott-Aldrich syndrome
. | HLA-identical sibling donor (n = 55) . | Other related donor (n = 48) . | Unrelated donor (n = 67) . | P . |
---|---|---|---|---|
Age at BMT, median (range), y | 3 (< 1-16) | 2 (< 1-17) | 2 (< 1-32) | < .05 |
Age at BMT | .26 | |||
Younger than 1 y | 13/55 (24%) | 12/48 (25%) | 9/67 (13%) | |
1-2 y | 18/55 (33%) | 26/48 (54%) | 34/67 (51%) | |
3-4 y | 10/55 (18%) | 3/48 (6%) | 9/67 (13%) | |
5-9 y | 7/55 (13%) | 3/48 (6%) | 8/67 (12%) | |
10 y or older | 7/55 (13%) | 4/48 (8%) | 7/67 (10%) | |
Lansky/Karnofsky performance score before BMT ≥ 90% | 26/45 (58%) | 28/47 (60%) | 43/63 (68%) | .48 |
Infection in the 2 wk before BMT | 12/52 (23%) | 16/48 (33%) | 11/61 (18%) | .18 |
Donor-recipient HLA mismatch | < .001 | |||
0 antigen mismatch | 55/55 (100%) | 3/43 (7%) | 43/62 (69%) | |
1 antigen mismatch | NA | 16/43 (37%) | 18/62 (29%) | |
≥ 2 antigen mismatch | NA | 24/43 (56%) | 1/62 (2%) | |
Male donor | 23/51 (45%) | 32/48 (67%) | 35/64 (55%) | .10 |
Year of transplant | < .001 | |||
1968-1979 | 5/55 (9%) | 2/48 (4%) | 0/67 (0%) | |
1980-1984 | 8/55 (15%) | 10/48 (21%) | 0/67 (0%) | |
1985-1989 | 13/55 (24%) | 13/48 (27%) | 3/67 (4%) | |
1990-1993 | 14/55 (25%) | 19/48 (40%) | 35/67 (52%) | |
1994-1996 | 15/55 (27%) | 4/48 (8%) | 29/67 (43%) | |
TBI for conditioning pretransplant | 4/55 (7%) | 11/45 (24%) | 14/64 (22%) | < .05 |
GVHD prophylaxis | < .001 | |||
MTX + CsA | 20/55 (36%) | 14/48 (29%) | 37/64 (58%) | |
MTX ± other | 19/55 (35%) | 2/48 (4%) | 3/64 (5%) | |
CsA ± other | 14/55 (25%) | 3/48 (6%) | 13/64 (20%) | |
T-cell depletion ± other | 0/55 (0%) | 26/48 (54%) | 11/64 (17%) | |
Other (non–T-cell depleted) or none* | 2/55 (4%) | 3/48 (6%) | 0/64 (0%) |
. | HLA-identical sibling donor (n = 55) . | Other related donor (n = 48) . | Unrelated donor (n = 67) . | P . |
---|---|---|---|---|
Age at BMT, median (range), y | 3 (< 1-16) | 2 (< 1-17) | 2 (< 1-32) | < .05 |
Age at BMT | .26 | |||
Younger than 1 y | 13/55 (24%) | 12/48 (25%) | 9/67 (13%) | |
1-2 y | 18/55 (33%) | 26/48 (54%) | 34/67 (51%) | |
3-4 y | 10/55 (18%) | 3/48 (6%) | 9/67 (13%) | |
5-9 y | 7/55 (13%) | 3/48 (6%) | 8/67 (12%) | |
10 y or older | 7/55 (13%) | 4/48 (8%) | 7/67 (10%) | |
Lansky/Karnofsky performance score before BMT ≥ 90% | 26/45 (58%) | 28/47 (60%) | 43/63 (68%) | .48 |
Infection in the 2 wk before BMT | 12/52 (23%) | 16/48 (33%) | 11/61 (18%) | .18 |
Donor-recipient HLA mismatch | < .001 | |||
0 antigen mismatch | 55/55 (100%) | 3/43 (7%) | 43/62 (69%) | |
1 antigen mismatch | NA | 16/43 (37%) | 18/62 (29%) | |
≥ 2 antigen mismatch | NA | 24/43 (56%) | 1/62 (2%) | |
Male donor | 23/51 (45%) | 32/48 (67%) | 35/64 (55%) | .10 |
Year of transplant | < .001 | |||
1968-1979 | 5/55 (9%) | 2/48 (4%) | 0/67 (0%) | |
1980-1984 | 8/55 (15%) | 10/48 (21%) | 0/67 (0%) | |
1985-1989 | 13/55 (24%) | 13/48 (27%) | 3/67 (4%) | |
1990-1993 | 14/55 (25%) | 19/48 (40%) | 35/67 (52%) | |
1994-1996 | 15/55 (27%) | 4/48 (8%) | 29/67 (43%) | |
TBI for conditioning pretransplant | 4/55 (7%) | 11/45 (24%) | 14/64 (22%) | < .05 |
GVHD prophylaxis | < .001 | |||
MTX + CsA | 20/55 (36%) | 14/48 (29%) | 37/64 (58%) | |
MTX ± other | 19/55 (35%) | 2/48 (4%) | 3/64 (5%) | |
CsA ± other | 14/55 (25%) | 3/48 (6%) | 13/64 (20%) | |
T-cell depletion ± other | 0/55 (0%) | 26/48 (54%) | 11/64 (17%) | |
Other (non–T-cell depleted) or none* | 2/55 (4%) | 3/48 (6%) | 0/64 (0%) |
BMT indicates bone marrow transplant; HLA, human leukocyte antigen; TBI, total body irradiation; GVHD, graft-versus-host disease; MTX, methotrexate; CsA, cyclosporine A.
One patient with an HLA-identical sibling donor and 3 patients with other related donors received no GVHD prophylaxis.