TABLE 1.
Categories | All patients | Subset at risk for CMV, R(+) and R(x), with CMV post-HSCT | Subset at risk for CMV, R(+) and R(x), without CMV post-HSCT | P-value |
---|---|---|---|---|
N | 90 | 18 | 62 | |
Age at transplant, median months (IQR) | 10 (3,43) | 42 (8,97) | 6 (3,17) | |
Biological sex | ||||
Male | 62 (69%) | 12 (67%) | 43 (69%) | .83 |
Female | 28 (31%) | 6 (33%) | 19 (31%) | |
Diagnosis† | ||||
TINF2 mutation | 1 (1%) | 0 (0%) | 1 (2%) | .11 |
CID | 9 (10%) | 4 (22%) | 4 (6%) | |
CID with syndromic features | 15 (17%) | 2 (11%) | 12 (19%) | |
Congenital defects of phagocytes | 3 (3%) | 1 (6%) | 0 (0%) | |
PIRD | 21 (23%) | 5 (28%) | 10 (17%) | |
SCID | 41 (46%) | 6 (33%) | 35 (56%) | |
Donor relation | ||||
Matched unrelated | 42 (47%) | 7 (39%) | 29 (47%) | .55 |
Matched related | 48 (53%) | 11 (61%) | 33 (53%) | |
Source of cells | ||||
BM | 44 (49%) | 8 (44%) | 28 (45%) | .99 |
Cord Blood | 5 (6%) | 1 (6%) | 4 (7%) | |
PBSC | 41 (45%) | 9 (50%) | 30 (48%) | |
ABO Incompatibility | ||||
No | 52 (58%) | 11 (61%) | 37 (60%) | .85 |
Major | 16 (18%) | 3 (17%) | 12 (19%) | |
Bidirectional | 2 (2%) | 0 (0%) | 2 (3%) | |
Minor | 20 (22%) | 4 (22%) | 11 (18%) | |
Alemtuzumab | 50 (56%) | 7 (39%) | 33 (53%) | .28 |
Conditioning | ||||
None | 15 (17%) | 3 (17%) | 12 (19%) | .94 |
NMAC | 39 (43%) | 9 (50%) | 28 (45%) | |
MAC | 36 (40%) | 6 (33%) | 22 (36%) | |
GVHD prophylaxis | ||||
None | 29 (32%) | 5 (28%) | 24 (39%) | .34 |
CNI + MTX | 39 (43%) | 8 (44%) | 25 (40%) | |
CNI + MMF | 7 (8%) | 3 (17%) | 3 (5%) | |
CNI + Others | 15 (17%) | 2 (11%) | 10 (16%) | |
T-cell depletion | 33 (37%) | 6 (33%) | 27 (44%) | .44 |
T-cell depletion 33 (37%) 6 (33%) 27 (4 4%) .44
Note: Patients characteristics and risk for CMV infection post-HSCT: overall column with characteristics of all 90 patients: other columns with univariate analysis performed only for subset of recipients at risk of CMV infection, R(+) and R(x).
Abbreviations: BM, bone marrow; CID, combined immunodeficiency; CMV, cy tomegalovirus; CsA, cyclosporin A; GVHD, graft-versus-host disease; HSCT, hematopoietic stem cell transplant; IQR, interquar tile range;MAC, myeloablative conditioning; MMF, mycophenolate mofetil; MT X, methotrexate, CNI, calcineurin inhibitors; NMAC, non-myeloablative conditioning; PBSC, peripheral blood stem cells; PIRD, primar y immune regulatory disorder; R(+), CMV seropositive; R(x) unknown CMV serostatus; SCID, severe combined immunodeficiency.
Based on IUIS categories of PIDD.