Table 9 Case reports of patients with atypical hemolytic uremic syndrome on their native kidneys treated with eculizumab
Reference | Mutation | Age at onset of HUS, evolution and response to plasmatherapy | Age at eculizumab initiation | Response to plasmatherapy of HUS episode at eculizumab initiation | Serum creatinine level at eculizumab initiation | Delay of hematological and renal improvement after initiation of eculizumab | Delay until complete remission of HUS after initiation of eculizumab | Protocol | Evolution of HUS Last Screat (follow-up under eculizumab) |
|---|---|---|---|---|---|---|---|---|---|
Gruppo et al 2009 [155] R. Gruppo PC | NI | < 8 days 3 relapses over 11 m, PI sensitive | 19 m | PE resistant | 265 μmol/L | 2 days | 10 days | Complete protocol | Remission 35 μmol/L (2 y 4 m) |
Fremont et al 2009 [156] | CFH | 4 y | 4 y | PE partially sensitive | 80 μmol/L | ND | 2 weeks | Complete protocol | Remission 26 μmol/L (10 weeks) |
Mache et al 2009 [157] | NI | 17.8 y PE sensitive 3 relapses at PE tapering | 17.11 y | PE resistant | 690 μmol/L | ~ 3 days (platelets increase) | 5 days (hematologic remission) | Single dose | Relapse at 2w ESRF |
Kose et al 2010 [158] | NI | 18 y | 18 y | PE resistant | ~310 μmol/L | 1 day | ~ 7 days | Single dose | Relapse at 2m ESRF |
Lapeyraque et al 2010 [159] AL. Lapeyraque PC | CFH S1191L V1197A | 7 m 11 relapses over 5.4 y, PE/PI sensitive | 6 y | PI resistant | 108 μmol/L | A few days | 1 week | Complete protocol | Remission 44 μmol/L (1 y 3 m) |
Prescott et al 2010 [160] HC. Prescott PC | CFI p.A258T | 47 y PE sensitive Relapse 2 w after PE cessation | 47 y | PE resistant | 610 μmol/L | - 7 days (Screat decrease) - 49 days (platelets normalization) | ~1.5 m | Complete protocol | Remission 230 μmol/L (7 m) |
Ohanian et al 2011 [161] | ND | 50 y | 50 y | No plasmatherapy | 600 μmol/L | - 4 days (LDH decrease) - 10 days (Screat decrease) | ~ 1.5 m | Complete protocol | Remission 198 μmol/L (2.5 m) |