Table 5.
Patient | Transplant | Age at transplantation | Time after first presentation of aHUS | HLA mismatch | Treatment before transplantation | Induction immunosuppression | Maintenance immunosuppression | Outcome |
---|---|---|---|---|---|---|---|---|
4a | DCD (en bloc) | 8 yr, 9 mo | 5 yr | Unknown | None | Unknown | Unknown | Primary nonfunction |
4a | LRTb ABOi |
9 yr, 3 mo | 5 yr, 6 mo | 1:1:1 | Rituximab and immunoadsorptionc | Basiliximab | Tacrolimus, MMF, prednisolone | Immediate recurrence,d eculizumab, creatinine: 72 μmol/l, eGFR: 47.6 ml/min per 1.73 m2 (follow-up 9 mo) |
5 | DBD | 12 yr, 6 mo | 1 yr, 10 mo | 1:1:0 | None | Basiliximab | Tacrolimus, azathioprine, prednisolone | Transplant failed after 7 yr 6 mo (severe AMR postpartum) |
12 | DBD | 10 yr | 1 yr, 5 mo | Unknown | None | Basiliximab | MMF, prednisolone | Creatinine: 80 μmol/l, eGFR: >60 ml/min per 1.73 m2 (follow-up 6 yr 9 mo) |
19 | DBD | 11 yr, 5 mo | 1 yr, 5 mo | 1:1:0 | None | Unknown | Tacrolimus, azathioprine, prednisolone | Creatinine: 109 μmol/l, eGFR: 48.4 ml/min per 1.73 m2 (follow-up: 3 yr 11 mo) |
22 | DCD | 9 yr, 9 mo | 2 yr | Unknown | None | Basiliximab | Tacrolimus (purine antagonist stopped) | Creatinine: 83 μmol/l, eGFR: >60 ml/min per 1.73 m2 (follow-up: 2 yr 5 mo) |
ABOi, ABO incompatible; aHUS, atypical hemolytic uremic syndrome; AMR, antibody-mediated rejection; DBD, donor after brain death; DCD, donor after cardiac death; eGFR, estimated glomerular filtration rate; LRT, living related transplant; MMF, mycophenolate mofetil.
eGFR by Schwartz formula (all patients were younger than 18 years of age at last follow-up).
Concomitant functionally significant CFI mutation.
LRT: living donor underwent genetic screening; no rare genetic variant, 0 copies CFHR1, 1 copy CFHR3.
Treatment given because of ABO incompatibility: rituximab 1 month before transplant, and immunoadsorption ×4 (anti–A titers reduced from 1:128 to 1:8).
Recurrence 9 hours after transplantation. Successfully treated with eculizumab.