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Table 10 Case reports of patients with post-transplant recurrence of atypical hemolytic uremic syndrome treated with eculizumab

From: Atypical hemolytic uremic syndrome

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)
  1. 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