Table 5. Clinical outcome and cell -mediated and humoral-immune reconstitution after BMT in 10 RAG2-deficient SCID patients.
Patient | months post BMT | IVIG | ALC | CD3 | CD4 | CD8 | CD56 | CD20 | PHA -SI | IGG | IGA | IGM | Chimerism in lymph (%) | Outcome |
P1 | 24 | yes | 632 | 430 | 152 | 316 | 90 | 6 | 2.6% | 486 | 19 | 27 | 30 | Died |
P2 | 24 | yes | 1840 | 1564 | 846 | 644 | 60 | 18 | 62% | ND | ND | ND | 34 | Died |
P3 | 76 | yes | 873 | 306 | 279 | 105 | 20 | 61 | 35% | 480 | UD | UD | 100 | Alive, cGVHD |
P4 | 72 | No | 2134 | 1643 | 597 | 1003 | 105 | 192 | 39% | 642 | 118 | 96 | 50 | Alive, cGVHD |
P5 | 120 | yes | 2346 | 1947 | 1173 | 868 | 115 | 47 | 50% | 2180 | 63 | 401 | 100 | Alive, cGVHD |
P6 | 6 | No | 1212 | 720 | 480 | 300 | 40 | 120 | 260% | ND | ND | ND | 80 | Alive and Well |
P7 | 34 | No | 4506 | 3154 | 1262 | 1622 | 450 | 541 | 89% | ND | ND | ND | 100 | Alive and Well |
P8 | 30 | No | 5084 | 3711 | 2695 | 1118 | 200 | 763 | 86% | 1120 | 75 | 78 | 100 | Alive and Well |
P9 | 3 | yes | 747 | 234 | 112 | 127 | 350 | 3 | ND | 1110 | UD | 20 | 100 | Alive and Well |
P10 | 8 | yes | 2702 | 1973 | 598 | 1189 | 270 | 162 | ND | 438 | UD | 18 | 100 | Alive and Well |
ND-not done, UD-undetectable, PHA – phytohemagglutinin, SI – stimulation index (patient/control), ALC - absolute lymphocyte count.