Characteristics of patients, details of transplantation, and outcome of immunologic reconstitution
| Patient characteristics . | Details of transplantation . | Stem cell graft . | T-cell reconstitution . | Chimerism . | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| UPN/sex . | SCID variant/ Gen-Def . | Age (mo) . | Stem cell donor . | HLA-MM . | CR . | Source . | T-cell depletion . | Nucleated cells 106/kg . | CD34+ cells 106/kg . | CD3+ cells 106/kg . | CD3 > 500/μL . | PHA > 100 SI . | MLR > 10 SI . | T cells . | B cells . | Monocytes . |
| day after transplantation . | ||||||||||||||||
| 314/M | B+/nd | 12 | Sister | 0 | No | BM | No | 2000 | nd | nd | 17 | 53 | 53 | D | nd | nd |
| 304/F | B+/nd | 6 | Sister | 0 | No | BM | No | 900 | nd | nd | 10 | 18 | 18 | D | nd | nd |
| 58/M | B+/nd | 39 | Brother | 0 | No | BM | No | 240 | nd | nd | 57 | 32 | 57 | D | nd | nd |
| 281/F | B+/nd | 2 | Mother | 3 | No | PBPC | CD34+CD2/3− | 27 | 27 | < 0.01 | 105 | 92 | 120 | D | R | R |
| 273/M | B+/yc | 8 | Mother | 1 | No | PBPC | CD34–CD2/3- | 24 | 24 | < 0.01 | 133 | 79 | 120 | D | R | R |
| 88/M | B+/yc | 1 | Mother | 2 | No | BM | SBA-ER− | 90 | nd | < 0.09 | 133 | 109 | 281 | D | R | R |
| 84/M | B+/yc | 1 | Father | 3 | No | BM | SBA-ER− | 60 | nd | < 0.06 | 124 | 124 | 168 | D | R | R |
| 346/M | B+/yc | 3 | Father | 3 | Bu | PBPC | CD34+CD2/3− | 13 | 12.6 | 0.02 | 155 | 155 | 155 | D | D/R | D/R |
| 328/M | B+/yc | 2 | Mother | 3 | Bu, Cy | PBPC | CD34+CD2/3− | 5 | 5 | < 0.01 | 166 | 78 | 112 | D | D | D |
| 271/M | B+/nd | 14 | Father | 3 | Bu, Cy | PBPC | CD34+CD2/3− | 33 | 33 | < 0.01 | 228 | 193 | 228 | D | D/R | D/R |
| Patient characteristics . | Details of transplantation . | Stem cell graft . | T-cell reconstitution . | Chimerism . | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| UPN/sex . | SCID variant/ Gen-Def . | Age (mo) . | Stem cell donor . | HLA-MM . | CR . | Source . | T-cell depletion . | Nucleated cells 106/kg . | CD34+ cells 106/kg . | CD3+ cells 106/kg . | CD3 > 500/μL . | PHA > 100 SI . | MLR > 10 SI . | T cells . | B cells . | Monocytes . |
| day after transplantation . | ||||||||||||||||
| 314/M | B+/nd | 12 | Sister | 0 | No | BM | No | 2000 | nd | nd | 17 | 53 | 53 | D | nd | nd |
| 304/F | B+/nd | 6 | Sister | 0 | No | BM | No | 900 | nd | nd | 10 | 18 | 18 | D | nd | nd |
| 58/M | B+/nd | 39 | Brother | 0 | No | BM | No | 240 | nd | nd | 57 | 32 | 57 | D | nd | nd |
| 281/F | B+/nd | 2 | Mother | 3 | No | PBPC | CD34+CD2/3− | 27 | 27 | < 0.01 | 105 | 92 | 120 | D | R | R |
| 273/M | B+/yc | 8 | Mother | 1 | No | PBPC | CD34–CD2/3- | 24 | 24 | < 0.01 | 133 | 79 | 120 | D | R | R |
| 88/M | B+/yc | 1 | Mother | 2 | No | BM | SBA-ER− | 90 | nd | < 0.09 | 133 | 109 | 281 | D | R | R |
| 84/M | B+/yc | 1 | Father | 3 | No | BM | SBA-ER− | 60 | nd | < 0.06 | 124 | 124 | 168 | D | R | R |
| 346/M | B+/yc | 3 | Father | 3 | Bu | PBPC | CD34+CD2/3− | 13 | 12.6 | 0.02 | 155 | 155 | 155 | D | D/R | D/R |
| 328/M | B+/yc | 2 | Mother | 3 | Bu, Cy | PBPC | CD34+CD2/3− | 5 | 5 | < 0.01 | 166 | 78 | 112 | D | D | D |
| 271/M | B+/nd | 14 | Father | 3 | Bu, Cy | PBPC | CD34+CD2/3− | 33 | 33 | < 0.01 | 228 | 193 | 228 | D | D/R | D/R |
UPN indicates unique patient number; SCID, severe combined immunodeficiency disease; Gen-Def, underlying genetic defect; HLA-MM, number of human leukocyte antigen mismatches; CR, conditioning regimen; PHA, phytohemagglutinin stimulation; SI, stimulatory index; MLR, mixed lymphocyte reaction; nd, not determined; yc, mutation of common-y-chain; Bu, busulfan 8 mg/kg; Cy, cyclophosphamide 200 mg/kg; BM, bone marrow; PBPC, peripheral blood progenitor cells; CD34+CD2/3−, PBPC after positive selection for CD34+ cells followed by E-rosetting as described in reference 9; SBA-ER−, T-cell-depleted bone marrow by lectin agglutination and E-rosetting, as described in Schreiner et al8; ne, not evaluated; D, donor; R, recipient.