Characteristics of hematopoietic cell transplantation, lymphoid chimerism, and humoral immune reconstitution in 33 SCID patients
Patient no. . | HCT . | Time post-HCT, y . | Chimerism, % . | Humoral reconstitution . | Group . | ||||||
---|---|---|---|---|---|---|---|---|---|---|---|
Donor . | CR . | T . | B . | B cells (× 109/L) . | Serum IgM, mg/mL . | Serum IgA, mg/mL . | Ig RT . | Ab response* . | |||
1a | MSD | None | 17.0 | 100 | 0 | 0.13 | 0.66 | 0.18 | No | Poor | 2 |
1b | MSD | None | 12.0 | 100 | 0 | 0.45 | 1.07 | 0.95 | No | − | 2 |
2a | 5/6-Ag MSD | Flu ATG | 12.0 | 100 | 100 | 0.86 | 3.16 | 0.88 | No | + | 1 |
2b | 5/6-Ag MSD | None | 10.0 | 100 | 21 | 0.3 | 2.63 | 1.25 | No | + | 1 |
3 | PIRD | Treo Flu Camp | 0.5 | 100 | 100 | 0.38 | 1.6 | 0.46 | Yes | n.a. | 1 |
4 | PIRD | Cy Flu ATG | 8.0 | 100 | 100 | 0.33 | n.a | No | + | 1 | |
5 | MMRD | Bu Cy ATG | 8.0 | 100 | 0 | 0.24 | 0.73 | < 0.06 | Yes | n.a. | 2 |
6 | MMRD | None | 15.0 | 100 | 0 | 0.14 | 0.18 | < 0.07 | Yes | n.a. | 2 |
7 | MMRD | Flu ATG | 18.0 | 100 | 100 | 0.21 | 1.33 | 4.12 | No | + | 1 |
8 | MMRD | Flu | 12.0 | 100 | 0 | 0.2 | 1.4 | 0.6 | Yes | n.a. | 2 |
9 | MMRD | None | 10.0 | 98 | 5 | 0.44 | 0.99 | < 0.07 | Yes | n.a. | 2 |
10 | MMRD | None | 4.1 | 95 | 1 | 0.81 | 0.72 | < 0.07 | Yes | − | 2 |
11a | MMRD | None | 5.5 | 97 | 1 | 0.10 | 0.04 | < 0.07 | Yes | − | 2 |
11b | MMRD | None | 5.5 | 97 | 1 | 0.11 | 0.18 | 0.12 | Yes | − | 2 |
12 | MMRD | Bu Cy | 23.0 | 100 | 98 | 0.13 | 1.85 | 2.19 | No | + | 1 |
13 | MMRD | None | 3.1 | 97 | 0 | 0.90 | 0.25 | < 0.07 | Yes | − | 2 |
14 | MMRD | Bu8 Cy | 9.8 | 100 | 34 | 0.21 | 3.58 | < 0.3 | Yes | − | 1 |
15 | MMRD | Flu | 4.0 | 100 | 100 | 0.59 | 1.43 | 0.46 | No | + | 1 |
16 | MMRD | None | 22.3 | 97 | 0 | 0.09 | 0.78 | < 0.07 | Yes | n.a. | 2 |
17 | MMRD | Bu Cy ATG | 6.3 | 100 | 100 | 0.22 | 1.09 | 1.27 | No | + | 1 |
18 | MMRD | None | 18.5 | 100 | 0 | 0.05 | < 0.04 | < 0.06 | Yes | − (isohemagglutinins) | 2 |
19 | MMRD | None | 11.25 | 100 | 0 | 0.42 | 1.9 | < 0.06 | Yes | n.a. | 2 |
20 | MMRD | None | 15.7 | 100 | 0 | 0.29 | 0.38 | < 0.1 | Yes | n.a. | 2 |
21a | MMRD | Bu 8 Cy | 8.2 | 100 | 15 | 3.2 | 2.9 | No | + | 1 | |
21b | MMRD (no TCD) | None | 1.7 | 100 | 0 | 0.76 | < 0.3 | Yes | n.a. | 2 | |
22 | MMRD | TT, Cy, ATG | 12.3 | 100 | 100 | 0.42 | 2.18 | 0.92 | No | + | 1 |
23 | UCB | Bu Cy Flu ATG | 7.0 | 100 | 100 | 1.78 | 0.89 | 0.32 | No | + | 1 |
24 | UCB | Bu Cy ATG | 2.0 | 100 | 100 | 2.69 | 2.39 | 0.97 | No | + | 1 |
25 | MSD | None | 12.2 | 97 | 11 | na | 1.18 | 1.50 | No | + | n.a. |
26 | MMRD | Anti-CD20 | 2.4 | 94 | 2 | na | 1.05 | < 0.07 | Yes | − | n.a. |
27 | MMRD | Cy ATG | 17.0 | 95 | 1 | na | 0.83 | 2.47 | No | + | n.a. |
28 | MSD | None | 3.5 | 90 | 50 | na | 0.2 | 0.35 | Yes | n.a. | n.a. |
29 | MUD | Bu Cy ATG | 4.5 | 99 | 98 | 0.03 | 0.89 | 0.97 | No | + | n.a. |
Patient no. . | HCT . | Time post-HCT, y . | Chimerism, % . | Humoral reconstitution . | Group . | ||||||
---|---|---|---|---|---|---|---|---|---|---|---|
Donor . | CR . | T . | B . | B cells (× 109/L) . | Serum IgM, mg/mL . | Serum IgA, mg/mL . | Ig RT . | Ab response* . | |||
1a | MSD | None | 17.0 | 100 | 0 | 0.13 | 0.66 | 0.18 | No | Poor | 2 |
1b | MSD | None | 12.0 | 100 | 0 | 0.45 | 1.07 | 0.95 | No | − | 2 |
2a | 5/6-Ag MSD | Flu ATG | 12.0 | 100 | 100 | 0.86 | 3.16 | 0.88 | No | + | 1 |
2b | 5/6-Ag MSD | None | 10.0 | 100 | 21 | 0.3 | 2.63 | 1.25 | No | + | 1 |
3 | PIRD | Treo Flu Camp | 0.5 | 100 | 100 | 0.38 | 1.6 | 0.46 | Yes | n.a. | 1 |
4 | PIRD | Cy Flu ATG | 8.0 | 100 | 100 | 0.33 | n.a | No | + | 1 | |
5 | MMRD | Bu Cy ATG | 8.0 | 100 | 0 | 0.24 | 0.73 | < 0.06 | Yes | n.a. | 2 |
6 | MMRD | None | 15.0 | 100 | 0 | 0.14 | 0.18 | < 0.07 | Yes | n.a. | 2 |
7 | MMRD | Flu ATG | 18.0 | 100 | 100 | 0.21 | 1.33 | 4.12 | No | + | 1 |
8 | MMRD | Flu | 12.0 | 100 | 0 | 0.2 | 1.4 | 0.6 | Yes | n.a. | 2 |
9 | MMRD | None | 10.0 | 98 | 5 | 0.44 | 0.99 | < 0.07 | Yes | n.a. | 2 |
10 | MMRD | None | 4.1 | 95 | 1 | 0.81 | 0.72 | < 0.07 | Yes | − | 2 |
11a | MMRD | None | 5.5 | 97 | 1 | 0.10 | 0.04 | < 0.07 | Yes | − | 2 |
11b | MMRD | None | 5.5 | 97 | 1 | 0.11 | 0.18 | 0.12 | Yes | − | 2 |
12 | MMRD | Bu Cy | 23.0 | 100 | 98 | 0.13 | 1.85 | 2.19 | No | + | 1 |
13 | MMRD | None | 3.1 | 97 | 0 | 0.90 | 0.25 | < 0.07 | Yes | − | 2 |
14 | MMRD | Bu8 Cy | 9.8 | 100 | 34 | 0.21 | 3.58 | < 0.3 | Yes | − | 1 |
15 | MMRD | Flu | 4.0 | 100 | 100 | 0.59 | 1.43 | 0.46 | No | + | 1 |
16 | MMRD | None | 22.3 | 97 | 0 | 0.09 | 0.78 | < 0.07 | Yes | n.a. | 2 |
17 | MMRD | Bu Cy ATG | 6.3 | 100 | 100 | 0.22 | 1.09 | 1.27 | No | + | 1 |
18 | MMRD | None | 18.5 | 100 | 0 | 0.05 | < 0.04 | < 0.06 | Yes | − (isohemagglutinins) | 2 |
19 | MMRD | None | 11.25 | 100 | 0 | 0.42 | 1.9 | < 0.06 | Yes | n.a. | 2 |
20 | MMRD | None | 15.7 | 100 | 0 | 0.29 | 0.38 | < 0.1 | Yes | n.a. | 2 |
21a | MMRD | Bu 8 Cy | 8.2 | 100 | 15 | 3.2 | 2.9 | No | + | 1 | |
21b | MMRD (no TCD) | None | 1.7 | 100 | 0 | 0.76 | < 0.3 | Yes | n.a. | 2 | |
22 | MMRD | TT, Cy, ATG | 12.3 | 100 | 100 | 0.42 | 2.18 | 0.92 | No | + | 1 |
23 | UCB | Bu Cy Flu ATG | 7.0 | 100 | 100 | 1.78 | 0.89 | 0.32 | No | + | 1 |
24 | UCB | Bu Cy ATG | 2.0 | 100 | 100 | 2.69 | 2.39 | 0.97 | No | + | 1 |
25 | MSD | None | 12.2 | 97 | 11 | na | 1.18 | 1.50 | No | + | n.a. |
26 | MMRD | Anti-CD20 | 2.4 | 94 | 2 | na | 1.05 | < 0.07 | Yes | − | n.a. |
27 | MMRD | Cy ATG | 17.0 | 95 | 1 | na | 0.83 | 2.47 | No | + | n.a. |
28 | MSD | None | 3.5 | 90 | 50 | na | 0.2 | 0.35 | Yes | n.a. | n.a. |
29 | MUD | Bu Cy ATG | 4.5 | 99 | 98 | 0.03 | 0.89 | 0.97 | No | + | n.a. |
Flu indicates fludarabine; ATG, anti-thymocyte globulin; PIRD, phenotypically identical related donor; Treo, treosulfan; Camp, Compath (anti-CD52); n.a., not available; Bu, busulfan; Cy, cyclophsophamide; TCD, T-cell depletion; TT, thiotepa; UCB, umbilical cord blood; and MUD, matched unrelated donor.
− indicates undetectable Ag-specific Ab response; Poor, induced but not protective Ag-specific Ab response; and +, protective Ag-specific Ab responses. For P18, no isohemagglutinins were detected.