Table 10.
Case reports of patients with post-transplant recurrence of atypical hemolytic uremic syndrome treated with eculizumab
| Reference | Gene | Previous transplantations | Age and post-tx course before eculizumab initiation | Delay from recurrence to eculizumab initiation | Screat at eculizumab initiation μmol/L |
Delay until platelet increase/hemolysis resolution/Screat decrease under eculizumab | Protocol | Recurrence after eculizumab cessation (delay) |
Evolution under eculizumab Last Screat (follow-up under eculizumab) |
|---|---|---|---|---|---|---|---|---|---|
| Nurnberger 2009 [162] J.Nurnberger PC |
CFH Y475S |
1st tx: recurrence at 5 w, PE resistant, graft loss |
37 y, 2nd tx Recurrence at 6 w PE resistant |
5 days | 132 | 2 days/6 days/24 h | Single dose | Likely but not biopsy proven (21 m). Graft loss. |
/ |
| Chatelet 2009 [121] 2010 [163] V.Chatelet PC |
C3 R570Q |
1st tx: recurrence at 5 m, graft loss after 2 y |
43 y, 2nd tx Recurrence at 3 y PE sensitive/dependent |
14 m | 320 | A few days/a few days/~1 month | Complete protocol | NA | 2 recurrences of hemolytic anemia when injections delayed by 6-8 days 230 μmol/L (2 y 5 m) |
| Legault 2009 [164] |
ND | No | 34 y, 1st tx Recurrence at 1 m and 5 m, PE sensitive initially, then resistant |
9 m | 323 | ND/ND/4 weeks | Complete protocol | NA | Remission 238 μmol/L (6 m) |
| Davin 2010 [137] JC. Davin PC |
CFH S1191L |
1st tx: recurrence at day 3, graft loss 2nd tx under prophylactic PE: recurrence at 10 w, graft loss |
17 y, 3rd tx Preventive PE Recurrence at 4 m, rescue (PE intensification) Plasma intolerance at 10 m |
10 m | 131 | NA (in remission at eculizumab initiation) | Complete protocol | NA | Remission 130 μmol/L (1 y 10 m) |
| Larrea 2010 [165] M.Lozano PC |
NI | No | 22 y, 1st tx Recurrence at day 12 PE resistant |
9 days | 415 | 36 h/36 h/3 days | Single dose | Yes (11.5 m) Eculizumab resumed |
Remission 177 μmol/L (5 m) Graft loss after 2nd eculizumab cessation (humoral rejection) |
| Zuber 2010 [131] J. Zuber PC |
CFH | 1st tx: recurrence, graft loss | 24 y, 2nd tx Preventive PI/PE Recurrence at day 1 PE resistant |
4 days | 500 | 24 h/24 h/3 days | Complete protocol | NA | Remission 62 μmol/L (9 m) |
| Al-Akash 2010 [166] SI. Al-Akash PC |
C3 R570W |
1st tx: recurrence at 4 y, graft loss 2nd tx: recurrence at 2 m, graft loss |
15 y, 3rd tx Preventive PE Recurrence at 2 m, PE partially sensitive |
~20 days | 220 | A few days | Complete protocol | NA | Remission 115 μmol/L (1 y 5 m) |
| Zimmerhakl 2010 [167] M.Riedl PC |
CFH W1183C |
No | 10 y, 1st tx Preventive PE | Preventive eculizumab |
~ 45 | NA | Complete protocol | NA | No recurrence 44 μmol/L (2 y 1 m) |
| Weitz 2011 [168] |
CFH E1198 stop |
No | 7 y, 1st tx | Preventive eculizumab |
NA (dialysis) |
NA | Complete protocol | NA | No recurrence Normal Screat (7 m) |
| Nester 2011 [169] |
Hybrid CFH | No | 12 y, 1st tx Preventive PE |
Preventive eculizumab |
NA (dialysis) |
NA | Complete protocol | NA | No recurrence 80 μmol/L (4 m) |
Preventive: started before transplantation or before recurrence; PC: personal communication, with permission; Screatinine: serum creatinine; CFH: factor H; NI: none identified; Tx: transplantation; NI: none identified; ND: not documented; NA: not applicable; PE: plasma exchange